Trial Outcomes & Findings for An Open Label Extension Study to Evaluate Inhaled Treprostinil in Adult PH With ILD Including CPFE (NCT NCT02633293)

Last Updated: 2022-12-02

Results Overview

The intent of the 6MWD test is to evaluate exercise capacity associated with carrying out activities of daily living. Change in 6MWD from Baseline correlates with the current clinical standard for assessing patient functional status in the treatment of PH and is considered an objective measure of patient functional status. Subjects will be instructed to walk down a corridor at a comfortable speed as far as they can manage for 6 minutes. Distance \<500 meters suggests considerable exercise limitation; Distance 500-800 meters suggests moderate limitation; Distance \>800 meters (with no rests) suggests mild or no limitation. Peak exposure 6MWD will occur by conducting 6MWT within 10 to 60 minutes after the most recent dose of study drug dose.

Recruitment status

TERMINATED

Study phase

PHASE2/PHASE3

Target enrollment

243 participants

Primary outcome timeframe

Baseline through Week 124 in RIN-PH-202 (Combined data from parent study RIN-PH-201 [NCT02630316] and this open-label extension study RIN-PH-202)

Results posted on

2022-12-02

Participant Flow

Participant milestones

Participant milestones
Measure	Inhaled Treprostinil Open-label access Inhaled Treprostinil: Inhaled treprostinil (6 mcg/breath) administered four times daily and titrated up to a maximum of 12 breaths four times daily
Overall Study STARTED	243
Overall Study Received Inhaled Treprostinil in RIN-PH-202 (Safety Population)	242
Overall Study COMPLETED	70
Overall Study NOT COMPLETED	173

Reasons for withdrawal

Reasons for withdrawal
Measure	Inhaled Treprostinil Open-label access Inhaled Treprostinil: Inhaled treprostinil (6 mcg/breath) administered four times daily and titrated up to a maximum of 12 breaths four times daily
Overall Study Death	56
Overall Study Progressive Disease	12
Overall Study Adverse Event	29
Overall Study Withdrawal by Subject	41
Overall Study Protocol Violation	1
Overall Study Lost to Follow-up	1
Overall Study Study Terminated by Sponsor	24
Overall Study Withdrawal by Sponsor Prior to Dose Administration	1
Overall Study Lung Transplant	5
Overall Study Lack of Efficacy	2
Overall Study Principal Investigator Terminated Study Site	1

Baseline Characteristics

Only 229 subjects had a Baseline 6MWD

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure	Inhaled Treprostinil n=242 Participants Open-label access Inhaled Treprostinil: Inhaled treprostinil (6 mcg/breath) administered four times daily and titrated up to a maximum of 12 breaths four times daily
Age, Continuous	70.0 years n=242 Participants
Sex: Female, Male Female	116 Participants n=242 Participants
Sex: Female, Male Male	126 Participants n=242 Participants
Ethnicity (NIH/OMB) Hispanic or Latino	17 Participants n=242 Participants
Ethnicity (NIH/OMB) Not Hispanic or Latino	224 Participants n=242 Participants
Ethnicity (NIH/OMB) Unknown or Not Reported	1 Participants n=242 Participants
Race (NIH/OMB) American Indian or Alaska Native	3 Participants n=242 Participants
Race (NIH/OMB) Asian	8 Participants n=242 Participants
Race (NIH/OMB) Native Hawaiian or Other Pacific Islander	0 Participants n=242 Participants
Race (NIH/OMB) Black or African American	53 Participants n=242 Participants
Race (NIH/OMB) White	178 Participants n=242 Participants
Race (NIH/OMB) More than one race	0 Participants n=242 Participants
Race (NIH/OMB) Unknown or Not Reported	0 Participants n=242 Participants
Time Since Diagnosis	0.51 years n=242 Participants
Current Diagnosis Idiopathic interstitial pneumonia	108 Participants n=242 Participants
Current Diagnosis Chronic hypersensitivity pneumonitis	13 Participants n=242 Participants
Current Diagnosis Occupational lung disease	5 Participants n=242 Participants
Current Diagnosis Combined pulmonary fibrosis and emphysema	59 Participants n=242 Participants
Current Diagnosis Connective tissue disease	57 Participants n=242 Participants
6-Minute Walk Distance (6MWD)	270.0 meters n=229 Participants • Only 229 subjects had a Baseline 6MWD
N-terminal Pro-brain Natriuretic Peptide (NT-proBNP)	465.70 pg/mL n=240 Participants • Only 240 subjects had a Baseline NT-proBNP measurement
Pulmonary Vascular Resistance	5.100 Wood units n=242 Participants
Mean Pulmonary Artery Pressure	34.5 mmHg n=242 Participants
Pulmonary Capillary Wedge Pressure	10.0 mmHg n=242 Participants

PRIMARY outcome

Timeframe: Baseline through Week 124 in RIN-PH-202 (Combined data from parent study RIN-PH-201 [NCT02630316] and this open-label extension study RIN-PH-202)

Population: Values are combined data from the Safety Populations of parent study RIN-PH-201 (n=326; NCT02630316) and this open-label extension study RIN-PH-202 (n=243) as presented in the RIN-PH-202 CSR over the course of 124 weeks (16-week treatment in RIN-PH-201 and up to 108 weeks in RIN-PH-202). The number of subjects analyzed at each week decreased as only those remaining on RIN-PH-202 who had not discontinued early due to the reasons show in the Participant Flow section were evaluated.

Outcome measures

Outcome measures
Measure	Inhaled Treprostinil n=296 Participants Open-label access Inhaled Treprostinil: Inhaled treprostinil (6 mcg/breath) administered four times daily and titrated up to a maximum of 12 breaths four times daily
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 4	1.5 meters Interval -197.0 to 258.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 8	5.0 meters Interval -259.0 to 196.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 12	9.5 meters Interval -190.0 to 192.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 16	10.0 meters Interval -269.0 to 183.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 20	12.0 meters Interval -189.0 to 169.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 28	10.0 meters Interval -222.0 to 175.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 40	3.0 meters Interval -261.0 to 171.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 52	3.0 meters Interval -223.0 to 194.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 64	-2.0 meters Interval -218.0 to 178.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 76	-2.0 meters Interval -209.0 to 210.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 88	-5.0 meters Interval -180.0 to 185.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 100	-11.0 meters Interval -213.0 to 196.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 112	-24.0 meters Interval -183.0 to 216.0
Change in Peak 6-Minute Walk Distance (6MWD) From Baseline Week 124	-16.0 meters Interval -287.0 to 190.0

SECONDARY outcome

Timeframe: Baseline through Week 124 in RIN-PH-202 (Combined data from parent study RIN-PH-201 [NCT02630316] and this open-label extension study RIN-PH-202)

Population: RIN-PH-202 CSR over the course of 124 weeks (16-week treatment in RIN-PH-201 and up to 108 weeks in RIN-PH-202). The number of subjects analyzed at each week decreased as only those remaining on RIN-PH-202 who had not discontinued early due to the reasons show in the Participant Flow section were evaluated.

The N-terminal pro-BNP (NT-proBNP) serum concentration is a useful biomarker associated with changes in right heart morphology and function. NT-proBNP serum concentration will be assessed to compare the severity of heart failure at Baseline and each visit through Week 124. Blood for NT-proBNP assessment must be drawn prior to conducting the 6-minute walk test (6MWT).

Outcome measures

Outcome measures
Measure	Inhaled Treprostinil n=285 Participants Open-label access Inhaled Treprostinil: Inhaled treprostinil (6 mcg/breath) administered four times daily and titrated up to a maximum of 12 breaths four times daily
Change in Plasma Concentration of N-terminal Pro-Brain Natriuretic Peptide (NT-proBNP) From Baseline Week 8	-5.10 pg/mL Interval -9704.2 to
Change in Plasma Concentration of N-terminal Pro-Brain Natriuretic Peptide (NT-proBNP) From Baseline Week 16	-1.30 pg/mL
Change in Plasma Concentration of N-terminal Pro-Brain Natriuretic Peptide (NT-proBNP) From Baseline Week 64	10.2 pg/mL Interval to 7306.0
Change in Plasma Concentration of N-terminal Pro-Brain Natriuretic Peptide (NT-proBNP) From Baseline Week 124	31.90 pg/mL Interval -4500.2 to

SECONDARY outcome

Timeframe: Baseline and Weeks 12, 48, and 108

Population: Subjects from the Placebo Group in parent study RIN-PH-201

Change in pulmonary function following inhaled treprostinil therapy will be measured by Forced Vital Capacity (FVC), calculated from a Pulmonary Function Test (PFT) performed at Baseline and Weeks 12, 48, and 108.

Outcome measures

Outcome measures
Measure	Inhaled Treprostinil n=121 Participants Open-label access Inhaled Treprostinil: Inhaled treprostinil (6 mcg/breath) administered four times daily and titrated up to a maximum of 12 breaths four times daily
Change in Percent Predicted Forced Vital Capacity (FVC) From Baseline Week 12	0.5 Percent Predicted FVC Interval -16.0 to 23.0
Change in Percent Predicted Forced Vital Capacity (FVC) From Baseline Week 48	2.0 Percent Predicted FVC Interval -10.0 to 21.0
Change in Percent Predicted Forced Vital Capacity (FVC) From Baseline Week 108	3.0 Percent Predicted FVC Interval -13.0 to 13.0

Adverse Events

Inhaled Treprostinil

Serious events: 133 serious events

Other events: 229 other events

Deaths: 56 deaths

Serious adverse events

Serious adverse events
Measure	Inhaled Treprostinil n=242 participants at risk Open-label access Inhaled Treprostinil: Inhaled treprostinil (6 mcg/breath) administered four times daily and titrated up to a maximum of 12 breaths four times daily
Blood and lymphatic system disorders Anaemia	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Blood and lymphatic system disorders Iron deficiency anaemia	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Blood and lymphatic system disorders Lymphadenitis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Blood and lymphatic system disorders Immune thrombocytopenia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Right ventricular failure	4.1% 10/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Cardiac failure congestive	2.1% 5/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Cardiac failure acute	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Cardio-respiratory arrest	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Acute left ventricular failure	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Atrial fibrillation	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Atrial flutter	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Bradycardia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Cardiac arrest	3.3% 8/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Cor pulmonale acute	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Coronary artery disease	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Coronary artery stenosis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Pulseless electrical activity	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Sinus node dysfunction	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Myocardial infarction	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Tachycardia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Abdominal pain upper	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Chronic gastritis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Constipation	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Diverticulum intestinal	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders haemorrhagic	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Gastrooesophageal reflux disease	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Haemorrhoidal haemorrhage	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Incarcerated umbilical hernia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Melaena	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Oesophageal ulcer	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Vomiting	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Duodenal ulcer haemorrhage	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Dysphagia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Gastrointestinal haemorrhage	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Lower gastrointestinal	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders haemorrhage	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Chest pain	1.7% 4/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Asthenia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Disease progression	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Non-cardiac chest pain	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Death	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Sudden cardiac death	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Hepatobiliary disorders Cholelithiasis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Hepatobiliary disorders Acute hepatic failure	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Pneumonia	8.7% 21/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Sepsis	2.9% 7/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Arthritis bacterial	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Bronchitis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations COVID-19 pneumonia	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Clostridium difficile infection	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Coccidioidomycosis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Fournier's gangrene	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Infective exacerbation of	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations bronchiectasis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Influenza	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Metapneumovirus infection	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Necrotising fasciitis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Pneumonia bacterial	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Pulmonary sepsis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Rhinovirus infection	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Septic shock	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Staphylococcal bacteraemia	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Urinary tract infection	2.1% 5/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Viral upper respiratory tract	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations infection	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations COVID-19	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Clostridium difficile colitis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Osteomyelitis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Pneumocystis jirovecii pneumonia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Pneumonia cytomegaloviral	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Pneumonia influenzal	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Pneumonia viral	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Respiratory syncytial virus	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Upper respiratory tract infection	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Urosepsis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Injury, poisoning and procedural complications Fall	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Injury, poisoning and procedural complications Subdural haematoma	1.7% 4/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Injury, poisoning and procedural complications Cervical vertebral fracture	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Injury, poisoning and procedural complications Femur fracture	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Injury, poisoning and procedural complications Hip fracture	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Injury, poisoning and procedural complications Spinal compression fracture	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Investigations Liver function test increased	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Investigations Transaminases increased	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Investigations Platelet count decreased	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Fluid overload	3.3% 8/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Hyperkalaemia	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Hypokalaemia	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Hyponatraemia	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Dehydration	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Diabetic ketoacidosis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Hypervolaemia	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Malnutrition	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Musculoskeletal and connective tissue disorders Arthritis	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Musculoskeletal and connective tissue disorders Back pain	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Musculoskeletal and connective tissue disorders Gouty arthritis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Neoplasms benign, malignant and unspecified (incl cysts and polyps) Bone cancer	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Neoplasms benign, malignant and unspecified (incl cysts and polyps) Follicular lymphoma	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Neoplasms benign, malignant and unspecified (incl cysts and polyps) Non-small cell lung cancer	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Neoplasms benign, malignant and unspecified (incl cysts and polyps) Squamous cell carcinoma of lung	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Neoplasms benign, malignant and unspecified (incl cysts and polyps) Uterine leiomyoma	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Neoplasms benign, malignant and unspecified (incl cysts and polyps) Chondrosarcoma	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Neoplasms benign, malignant and unspecified (incl cysts and polyps) Lung neoplasm malignant	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Neoplasms benign, malignant and unspecified (incl cysts and polyps) Small cell lung cancer	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Syncope	2.5% 6/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Carotid artery stenosis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Cerebrovascular accident	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Embolic stroke	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Hypoaesthesia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Ischaemic stroke	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Presyncope	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Thrombotic stroke	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Cerebral haemorrhage	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Encephalopathy	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Headache	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Seizure	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Renal and urinary disorders Acute kidney injury	3.7% 9/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Renal and urinary disorders Nephrolithiasis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Reproductive system and breast disorders Vaginal haemorrhage	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Acute respiratory failure	11.6% 28/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Respiratory failure	5.4% 13/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pulmonary hypertension	2.5% 6/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Dyspnoea	2.9% 7/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Interstitial lung disease	3.3% 8/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Chronic obstructive pulmonary disease	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Combined pulmonary fibrosis and emphysema	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Dyspnoea exertional	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Hypoxia	1.7% 4/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pulmonary embolism	1.2% 3/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Epistaxis	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Haemoptysis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Idiopathic pulmonary fibrosis	2.1% 5/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pneumothorax	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Chronic respiratory failure	1.7% 4/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Idiopathic interstitial pneumonia	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Lung cyst	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pleural effusion	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pneumomediastinum	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pneumothorax spontaneous	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pulmonary fibrosis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pulmonary oedema	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Respiratory arrest	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Respiratory distress	0.83% 2/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Skin and subcutaneous tissue disorders Intertrigo	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Skin and subcutaneous tissue disorders Subcutaneous emphysema	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Surgical and medical procedures Atrial appendage closure	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Vascular disorders Hypotension	1.7% 4/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Vascular disorders Deep vein thrombosis	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Vascular disorders Hypertension	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Vascular disorders Orthostatic hypotension	0.41% 1/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.

Other adverse events

Other adverse events
Measure	Inhaled Treprostinil n=242 participants at risk Open-label access Inhaled Treprostinil: Inhaled treprostinil (6 mcg/breath) administered four times daily and titrated up to a maximum of 12 breaths four times daily
Respiratory, thoracic and mediastinal disorders Cough	26.9% 65/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Dyspnoea	26.0% 63/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Headache	18.6% 45/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Diarrhoea	15.3% 37/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Dizziness	14.9% 36/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Upper respiratory tract infection	14.0% 34/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Nausea	13.2% 32/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Fatigue	13.2% 32/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Pneumonia	12.4% 30/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Acute respiratory failure	12.4% 30/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Urinary tract infection	11.2% 27/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Musculoskeletal and connective tissue disorders Back pain	10.7% 26/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Productive cough	10.3% 25/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Chest pain	9.9% 24/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Hypoxia	9.9% 24/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Musculoskeletal and connective tissue disorders Arthralgia	9.1% 22/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
General disorders Oedema peripheral	8.3% 20/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Nasopharyngitis	8.3% 20/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Oropharyngeal pain	7.9% 19/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Sinusitis	7.4% 18/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Interstitial lung disease	7.0% 17/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Nervous system disorders Syncope	6.6% 16/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Throat irritation	6.6% 16/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Infections and infestations Bronchitis	6.2% 15/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Cardiac disorders Right ventricular failure	5.8% 14/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Metabolism and nutrition disorders Fluid overload	5.8% 14/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Musculoskeletal and connective tissue disorders Pain in extremity	5.8% 14/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Renal and urinary disorders Acute kidney injury	5.8% 14/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Pulmonary hypertension	5.8% 14/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Gastrointestinal disorders Vomiting	5.4% 13/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Injury, poisoning and procedural complications Fall	5.4% 13/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Respiratory failure	5.4% 13/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Musculoskeletal and connective tissue disorders Muscle spasms	5.0% 12/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Dyspnoea exertional	5.0% 12/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.
Respiratory, thoracic and mediastinal disorders Nasal congestion	5.0% 12/242 • AEs were recorded throughout the course of the study from the time that each subject signed the ICF until all study assessments were completed, through the time that the study was terminated by the Sponsor (following the approval of inhaled treprostinil for use in patients with PH-ILD), up to 108 weeks. All AEs were followed until resolution (or return to normal or baseline values), until they were judged by the Investigator to no longer be clinically significant, or for at least 30 days if the AE extended beyond the final study visit. All SAEs were followed until resolution, death, or the subject was lost to follow-up, even if they were ongoing more than 30 days after completion of the final study visit.

Additional Information

Global Medical Information

United Therapeutics Corp.

Phone: 919-485-8350

Email: [email protected]

Results disclosure agreements

Principal investigator is a sponsor employee Institution and/or Principal Investigator agree not to publish or publicly present any results of the Study without the prior written consent of Sponsor, not to be unreasonably withheld or delayed. Institution and/or Principal Investigator further agree to provide Sponsor with drafts of any such publication or presentation for review and approval no less than 30 days prior to submission for publication or the date of public presentation.
Publication restrictions are in place

Restriction type: OTHER