Exploration of Metabolome in Patients With Interstitial Lung Disease and Pulmonary Hypertension With or Without Specific Pulmonary Hypertension Treatment

NCT ID: NCT07254338

Last Updated: 2026-01-06

Basic Information

• Recruitment Status: RECRUITING
• Total Enrollment: 80 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2026-01-01
• Study Completion Date: 2029-07-01

Brief Summary

Fibrosing interstitial lung diseases (FILDs) encompass a group of rare diseases characterized by progressive pulmonary fibrosis leading to respiratory failure. Current treatments primarily aim to slow disease progression but remain limited, making lung transplantation the ultimate recourse.

The development of pulmonary hypertension (PH) in the context of FILDs significantly worsens morbidity and mortality and drastically reduces patients' life expectancy. Conventional treatments for PH are generally ineffective in this setting. Nevertheless, some promising therapeutic agents are currently under investigation, particularly inhaled prostacyclin analogs such as treprostinil, which have demonstrated efficacy in recent clinical studies.

Our study aims to explore, in a minimally invasive manner, variations in metabolites in the serum and urine of patients with PH secondary to FILDs, before and during treatment. The main objective is to better understand the systemic effect of these treatments. Furthermore, the identification of metabolomic signatures will allow us to differentiate responders from non-responders, thus providing valuable prognostic and predictive criteria.

To date, some patients do not benefit from the available treatments, and better selection of responders could prevent iatrogenic effects in patients whose clinical condition is already fragile. In addition, characterizing the systemic mode of action of these treatments could pave the way for new clinical research focused on the profiles of responding patients.

Finally, a thorough understanding of the efficacy of the studied therapies is essential. Indeed, effective treatment of PH in the context of FILDs could not only slow disease progression but also reduce the need for lung transplantation, a major challenge in a context of organ shortage.

Conditions

Interstitial Lung Disease; Precapillar Pulmonary Hypertension

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

Group "intervention"

Patients who suffer from ILD and ILD-PH from this group will be given specific treatments (especially inhaled TREPROSTINIL, up to 3 breath per use; 4 uses a day) for ILD-PH after usual independent collegial consultation Those patients will have before and after treatment blood and urine metabolomic profile.

They will have baseline and follow up blood and urine sampling. They will be offer possibility to perform up to 6 auto-sampling at home.

Blood sampling for metabolomic profiling

Intervention Type: OTHER

Blood (veinous) will be collected under standard care conditions, in a dry red blood tube without additional gel (4 mL) to obtain serum after centrifugation for 10 minutes at 1500g, in the form of 500µL aliquots, labelled with the patient's code in the study.

This sampling will be performed at baseline in both group; and after follow up completion in Group 1 (4-6 month)

Urine sampling for metabolomic profiling

Intervention Type: OTHER

Urine collected from patient into a dry powder compact in the waiting room before or after the consultation. Preparation of two cryotubes containing at least 1 mL per sample, labelled with the patient's code in the study. In the absence of a calling point, strict aseptic conditions are not required, as the use of chemicals or antiseptic soaps can interfere with the dosages.

This sampling will be performed in Group 1 and 2 at baseline; and in Group 1 at 2-month and after completion of follow-up time (4-6 month).

Group " control "

Patients who suffer from ILD and ILD-PH from this group will not be given specific ILD-PH treatment based on insufficient haemodynamic data or clinical contraindications after independent collegial consultation.

They will have only baseline blood and urine sampling.

Blood sampling for metabolomic profiling

Intervention Type: OTHER

Blood (veinous) will be collected under standard care conditions, in a dry red blood tube without additional gel (4 mL) to obtain serum after centrifugation for 10 minutes at 1500g, in the form of 500µL aliquots, labelled with the patient's code in the study.

This sampling will be performed at baseline in both group; and after follow up completion in Group 1 (4-6 month)

Urine sampling for metabolomic profiling

Intervention Type: OTHER

Urine collected from patient into a dry powder compact in the waiting room before or after the consultation. Preparation of two cryotubes containing at least 1 mL per sample, labelled with the patient's code in the study. In the absence of a calling point, strict aseptic conditions are not required, as the use of chemicals or antiseptic soaps can interfere with the dosages.

This sampling will be performed in Group 1 and 2 at baseline; and in Group 1 at 2-month and after completion of follow-up time (4-6 month).

Interventions

Blood sampling for metabolomic profiling

Blood (veinous) will be collected under standard care conditions, in a dry red blood tube without additional gel (4 mL) to obtain serum after centrifugation for 10 minutes at 1500g, in the form of 500µL aliquots, labelled with the patient's code in the study.

This sampling will be performed at baseline in both group; and after follow up completion in Group 1 (4-6 month)

Intervention Type: OTHER

Urine sampling for metabolomic profiling

Urine collected from patient into a dry powder compact in the waiting room before or after the consultation. Preparation of two cryotubes containing at least 1 mL per sample, labelled with the patient's code in the study. In the absence of a calling point, strict aseptic conditions are not required, as the use of chemicals or antiseptic soaps can interfere with the dosages.

This sampling will be performed in Group 1 and 2 at baseline; and in Group 1 at 2-month and after completion of follow-up time (4-6 month).

Intervention Type: OTHER

Eligibility Criteria

Inclusion Criteria

• Patients suffering from progressive interstitial lung disease (ILD) and especially lung fibrosis
• Suspicion of precapillar pulmonary hypertension (group 3 PH / ILD-PH)
• Patients undergoing cardiac catheterisation for haemodynamic confirmation
• Patient who has given informed consent

Exclusion Criteria

• Patients suffering from other forms of PH (i.e. PAH, CTEPH, heart failure, multifactorial

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: Yes

Sponsors

Hospices Civils de Lyon

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Locations

Hôpital Cardiologique et Pneumologique Louis Pradel

Bron, Rhone, France

Site Status: RECRUITING

Countries

France

Central Contacts

Ségolène TURQUIER

Role: CONTACT

segolene.turquier@chu-lyon.fr

04 72 35 73 32 ext. +33

David LAVILLE

Role: CONTACT

david.laville@etu.univ-lyon1.fr

04 27 85 51 39 ext. +33

Facility Contacts

Ségolène TURQUIER

Role: primary

segolene.turquier@chu-lyon.fr

04 72 35 73 32 ext. +33

David LAVILLE

Role: backup

david.laville@chu-lyon.fr

04 27 85 51 39 ext. +33

Other Identifiers

69HCL25_0216

Identifier Type: -

Identifier Source: org_study_id