Trial Outcomes & Findings for A Pilot Study to Evaluate the Use of C1 Esterase Inhibitor (Human) in Patients With Acute Antibody-Mediated Rejection (NCT NCT01147302)

NCT ID: NCT01147302

Last Updated: 2021-06-11

Results Overview

The protocol-specified Day 20 (post-treatment) biopsy was compared to the qualifying biopsy to assess changes in histopathology for light and immunofluorescence microscopy. The Central Pathologist provided the following categorical information from the qualifying biopsy in an AMR Scorecard: C4d Score (0-100), Margination Score (0-100) Glomerulitis Score (0-100), Vasculitis Score (0-100), Glomerulosclerosis Score (0-100), Chronic Glomerulopathy Score (0-100), Interstitial Fibrosis Score (0-100), and the Chronic Vasculitis Score (0-100), with 0 being absence of abnormal histopathology. The "qualifying" renal allograft biopsy was performed as standard of care (SOC) within 12 months after transplant and prior to screening for this study. The first dose of study drug (Day 1) was administered within 72 hours after qualifying biopsy. A negative change from baseline indicates that histopathology has improved. Endpoint includes subjects with both Qualifying and Day 20 Biopsies.

Recruitment status

COMPLETED

Study phase

PHASE2

Target enrollment

18 participants

Primary outcome timeframe

Within 72 hours prior to first dose of study drug, Day 20

Results posted on

2021-06-11

Participant Flow

Participant milestones

Participant milestones
Measure
Placebo
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Overall Study
STARTED
9
9
Overall Study
COMPLETED
9
9
Overall Study
NOT COMPLETED
0
0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

A Pilot Study to Evaluate the Use of C1 Esterase Inhibitor (Human) in Patients With Acute Antibody-Mediated Rejection

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Total
n=18 Participants
Total of all reporting groups
Age, Continuous
48.8 years
STANDARD_DEVIATION 13.0 • n=5 Participants
48.6 years
STANDARD_DEVIATION 12.5 • n=7 Participants
48.7 years
STANDARD_DEVIATION 12.4 • n=5 Participants
Age, Customized
18 to 44 years
3 Participants
n=5 Participants
4 Participants
n=7 Participants
7 Participants
n=5 Participants
Age, Customized
45 to 64 years
4 Participants
n=5 Participants
4 Participants
n=7 Participants
8 Participants
n=5 Participants
Age, Customized
65 to 75 years
2 Participants
n=5 Participants
1 Participants
n=7 Participants
3 Participants
n=5 Participants
Sex: Female, Male
Female
6 Participants
n=5 Participants
5 Participants
n=7 Participants
11 Participants
n=5 Participants
Sex: Female, Male
Male
3 Participants
n=5 Participants
4 Participants
n=7 Participants
7 Participants
n=5 Participants

PRIMARY outcome

Timeframe: Within 72 hours prior to first dose of study drug, Day 20

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

The protocol-specified Day 20 (post-treatment) biopsy was compared to the qualifying biopsy to assess changes in histopathology for light and immunofluorescence microscopy. The Central Pathologist provided the following categorical information from the qualifying biopsy in an AMR Scorecard: C4d Score (0-100), Margination Score (0-100) Glomerulitis Score (0-100), Vasculitis Score (0-100), Glomerulosclerosis Score (0-100), Chronic Glomerulopathy Score (0-100), Interstitial Fibrosis Score (0-100), and the Chronic Vasculitis Score (0-100), with 0 being absence of abnormal histopathology. The "qualifying" renal allograft biopsy was performed as standard of care (SOC) within 12 months after transplant and prior to screening for this study. The first dose of study drug (Day 1) was administered within 72 hours after qualifying biopsy. A negative change from baseline indicates that histopathology has improved. Endpoint includes subjects with both Qualifying and Day 20 Biopsies.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Change From Baseline in Histopathology Endpoints
C4d score
-45.0 scores on a scale
Standard Deviation 46.9
-36.1 scores on a scale
Standard Deviation 33.4
Change From Baseline in Histopathology Endpoints
Margination score
-6.0 scores on a scale
Standard Deviation 14.0
12.6 scores on a scale
Standard Deviation 25.9
Change From Baseline in Histopathology Endpoints
Glomerulitis score
6.7 scores on a scale
Standard Deviation 26.6
2.7 scores on a scale
Standard Deviation 13.6
Change From Baseline in Histopathology Endpoints
Vasculitis score
-3.9 scores on a scale
Standard Deviation 7.8
3.2 scores on a scale
Standard Deviation 6.4
Change From Baseline in Histopathology Endpoints
Glomerulosclerosis score
-1.4 scores on a scale
Standard Deviation 7.8
-6.3 scores on a scale
Standard Deviation 7.9
Change From Baseline in Histopathology Endpoints
Chronic glomerulopathy score
0.2 scores on a scale
Standard Deviation 0.7
0 scores on a scale
Standard Deviation 0.0
Change From Baseline in Histopathology Endpoints
Interstitial fibrosis score
5.9 scores on a scale
Standard Deviation 9.8
11.6 scores on a scale
Standard Deviation 20.9
Change From Baseline in Histopathology Endpoints
Chronic vasculitis score
-1.7 scores on a scale
Standard Deviation 18.2
2.9 scores on a scale
Standard Deviation 9.0

SECONDARY outcome

Timeframe: 90 days after start of treatment

Population: The Intent-to-Treat Safety (ITT-S) population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

The "qualifying" renal allograft biopsy was performed as standard of care within 12 months after transplant and prior to screening. The qualifying biopsy was used to establish the diagnosis of AMR and was evaluated for all of the following to obtain baseline assessments: the presence of C4d, and monocyte or neutrophil infiltration around the peritubular capillaries (PTCs) and/or glomeruli. The Central Pathologist provided the following information from the qualifying biopsy in an AMR Scorecard: C4d Score, Glomerulitis Score, Vasculitis Score, Glomerulosclerosis Score, Chronic Glomerulopathy Score, Interstitial Fibrosis Score, and the Chronic Vasculitis Score. The Banff AMR Scoring System was used to summarize these scores. Resolution determination was made based on clinical criteria (improvement of serum creatinine ± decrease of DSA titer, and/or increase in urine output) and histopathology. The first dose of study drug (Day 1) was administered within 72 hours after qualifying biopsy.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Number of Participants With Resolution of The Qualifying Episode of Antibody-Mediated Rejection (AMR)
Clinical or histopathological resolution, n=9, 9
6 participants
7 participants
Number of Participants With Resolution of The Qualifying Episode of Antibody-Mediated Rejection (AMR)
Resolution based on clinical criteria, n=6, 7
3 participants
0 participants
Number of Participants With Resolution of The Qualifying Episode of Antibody-Mediated Rejection (AMR)
Resolution based on histopathology, n=6, 7
4 participants
7 participants

SECONDARY outcome

Timeframe: From Day 1 to Days 20 and 90

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

Graft function was assessed by measuring serum creatinine. Serum creatinine level was obtained directly from laboratory results. Baseline was the last value collected prior to first dose of study drug. A negative change from baseline indicates that serum creatinine levels have decreased. Values for Day 90 were collected +/= 14 days.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Change From Baseline in Serum Creatinine
Day 20, n=9,9
-0.2 mg/dL
Standard Deviation 0.6
-0.1 mg/dL
Standard Deviation 0.4
Change From Baseline in Serum Creatinine
Day 90, n=9,8
-0.1 mg/dL
Standard Deviation 0.6
-0.1 mg/dL
Standard Deviation 0.4

SECONDARY outcome

Timeframe: From Day 1 to Days 20 and 90

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

Graft function was assessed by measuring creatinine clearance. Creatinine clearance was calculated by the Cockcroft-Gault formula. Baseline was the last value collected prior to first dose of study drug. A positive change from baseline indicates that the clearance rate has increased. Values for Day 90 were collected +/= 14 days.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Change From Baseline in Creatinine Clearance
Day 20, n=9,9
11.4 mL/min
Standard Deviation 24.2
12.9 mL/min
Standard Deviation 26.2
Change From Baseline in Creatinine Clearance
Day 90, n=9,9
3.4 mL/min
Standard Deviation 17.2
8.1 mL/min
Standard Deviation 17.7

SECONDARY outcome

Timeframe: From Day 1 through Days 20 and 90

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

If necessary, rescue therapy included plasmapheresis. Participating centers used plasmapheresis for desensitization, if necessary, prior to transplant and also for the treatment of acute AMR. Plasmapheresis therapy was performed for the qualifying episode of AMR according to standards at the investigational site and at the discretion of the investigator. Sessions include those prior to first dose. If plasmapheresis therapy occurred on the same day as study drug dosing, study drug was administered after completion of the plasmapheresis session.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Number of Plasmapheresis Sessions
Day 1 through Day 20, n=9, 9
7.4 sessions
Standard Deviation 5.2
9.0 sessions
Standard Deviation 3.2
Number of Plasmapheresis Sessions
Day 1 through Day 90, n=7, 6
10.4 sessions
Standard Deviation 7.6
10.7 sessions
Standard Deviation 4.2

SECONDARY outcome

Timeframe: From Day 1 to Day 90

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

If necessary, rescue therapy included splenectomy.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Number of Participants Who Required Salvage Splenectomy
0 participants
0 participants

SECONDARY outcome

Timeframe: From Day 1 to Day 90

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Number of Deaths
0 participants
0 participants

SECONDARY outcome

Timeframe: From the day of enrollment to Day 90

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

Allograft failure was determined by the presence of the following criteria: current renal allograft nephrectomy and/or a clinical determination that the allograft irreversibly and irrevocably ceased functioning.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Number of Participants With Allograft Failure
0 participants
0 participants

SECONDARY outcome

Timeframe: Day 13 pre-plasmapheresis (if performed) and pre-dose then 0.5, 2, 24, 48, 96, 168 and 288 (optional) hours post start of infusion

Plasma samples were used for the determination of antigenic C1 INH concentrations. Primary pharmacokinetic (PK) parameters were calculated using baseline-corrected concentration-versus-time data following the last dose and noncompartmental techniques, as appropriate. The following PK parameters were calculated: baseline concentration (Cbaseline), maximum concentration (Cmax), average concentration at steady-state (Cav,ss), time to maximum concentration (tmax), minimum concentration (Cmin), area under the concentration-time curve from time zero to last quantifiable concentration at time t (AUC0-t). Blood was collected on Day 13 at the indicated timepoints. If plasmapheresis was performed on a dosing day, blood samples were obtained before plasmapheresis, prior to study drug administration (post-plasmapheresis), and at time points relative to the start of infusion.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Serum Concentrations of C1 Inhibitor (C1 INH) Antigen
Unadjusted Cbaseline, n=9, 9
0.149 g/L
Standard Deviation 0.035
0.115 g/L
Standard Deviation 0.041
Serum Concentrations of C1 Inhibitor (C1 INH) Antigen
Cmin, n=9, 9
0.131 g/L
Standard Deviation 0.0366
0.129 g/L
Standard Deviation 0.0462
Serum Concentrations of C1 Inhibitor (C1 INH) Antigen
Cmax, n=9, 9
0.014 g/L
Standard Deviation 0.020
0.081 g/L
Standard Deviation 0.033
Serum Concentrations of C1 Inhibitor (C1 INH) Antigen
Cavg, n=5, 9
0.012 g/L
Standard Deviation 0.014
0.052 g/L
Standard Deviation 0.037

SECONDARY outcome

Timeframe: Day 13 pre-plasmapheresis (if performed) and pre-dose then 0.5, 2, 24, 48, 96, 168 and 288 (optional) hours post start of infusion

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

Plasma samples were used for the determination of functional C1 INH concentrations. Primary pharmacokinetic (PK) parameters were calculated using baseline-corrected concentration-versus-time data following the last dose and noncompartmental techniques, as appropriate. The following PK parameters were calculated: baseline concentration (Cbaseline), maximum concentration (Cmax), average concentration at steady-state (Cav,ss), time to maximum concentration (tmax), minimum concentration (Cmin), area under the concentration-time curve from time zero to last quantifiable concentration at time t (AUC0-t). Blood was collected on Day 13 at the indicated timepoints. If plasmapheresis was performed on a dosing day, blood samples were obtained before plasmapheresis, prior to study drug administration (post-plasmapheresis), and at time points relative to the start of infusion.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Serum Concentrations of C1 INH Functional Activity
Cmax, n=9, 9
0.147 Units/mL
Standard Deviation 0.327
0.884 Units/mL
Standard Deviation 0.457
Serum Concentrations of C1 INH Functional Activity
Unadjusted Cbaseline, n=9, 9
1.24 Units/mL
Standard Deviation 0.592
0.777 Units/mL
Standard Deviation 0.301
Serum Concentrations of C1 INH Functional Activity
Cmin, n=9, 9
1.02 Units/mL
Standard Deviation 0.42
0.961 Units/mL
Standard Deviation 0.441
Serum Concentrations of C1 INH Functional Activity
Cavg, n=6, 8
0.205 Units/mL
Standard Deviation 0.403
0.711 Units/mL
Standard Deviation 0.513

SECONDARY outcome

Timeframe: Day 13 pre-plasmapheresis (if performed) and pre-dose then 0.5, 2, 24, 48, 96, 168 and 288 (optional) hours post start of infusion

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

Plasma samples were used for the determination of antigenic and functional C1 INH concentrations. Primary pharmacokinetic (PK) parameters were calculated using baseline-corrected concentration-versus-time data following the last dose and noncompartmental techniques, as appropriate. The following PK parameters were calculated: baseline concentration (Cbaseline), maximum concentration (Cmax), average concentration at steady-state (Cav,ss), time to maximum concentration (tmax), minimum concentration (Cmin), area under the concentration-time curve from time zero to last quantifiable concentration at time t (AUC0-t). Blood was collected on Day 13 at the indicated timepoints. If plasmapheresis was performed on a dosing day, blood samples were obtained before plasmapheresis, prior to study drug administration (post-plasmapheresis), and at time points relative to the start of infusion.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Time to Maximum Plasma Concentration (Tmax) of C1 INH
C1 INH antigen, n=5, 9
43.2 hours
Interval 0.0 to 44.9
0.55 hours
Interval 0.48 to 21.02
Time to Maximum Plasma Concentration (Tmax) of C1 INH
C1 INH functional activity, n=6, 9
4.07 hours
Interval 0.0 to 47.0
3.88 hours
Interval 0.55 to 45.0

SECONDARY outcome

Timeframe: Day 13 pre-plasmapheresis (if performed) and pre-dose then 0.5, 2, 24, 48, 96, 168 and 288 (optional) hours post start of infusion

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

Plasma samples were used for the determination of antigenic C1 INH parameters. Primary pharmacokinetic (PK) parameters were calculated using baseline-corrected concentration-versus-time data following the last dose and noncompartmental techniques, as appropriate. The following PK parameters were calculated: baseline concentration (Cbaseline), maximum concentration (Cmax), average concentration at steady-state (Cav,ss), time to maximum concentration (tmax), minimum concentration (Cmin), and area under the concentration-time curve from time zero to last quantifiable concentration at time t (AUC0-t). Blood was collected on Day 13 at the indicated timepoints. If plasmapheresis was performed on a dosing day, blood samples were obtained before plasmapheresis, prior to study drug administration (post-plasmapheresis), and at time points relative to the start of infusion.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Area Under The Concentration-Time Curve (AUC) of C1 INH Antigen
AUC0-48hours, n=5, 9
0.590 g*h/L
Standard Deviation 0.691
2.51 g*h/L
Standard Deviation 1.79
Area Under The Concentration-Time Curve (AUC) of C1 INH Antigen
AUClast, n=9, 9
2.24 g*h/L
Standard Deviation 4.19
8.64 g*h/L
Standard Deviation 9.50

SECONDARY outcome

Timeframe: Day 13 pre-plasmapheresis (if performed) and pre-dose then 0.5, 2, 24, 48, 96, 168 and 288 (optional) hours post start of infusion

Population: The ITT-S population, defined as participants who received at least 1 dose (or any portion of a dose) of study drug (CINRYZE or placebo).

Plasma samples were used for the determination of functional C1 INH parameters. Primary pharmacokinetic (PK) parameters were calculated using baseline-corrected concentration-versus-time data following the last dose and noncompartmental techniques, as appropriate. The following PK parameters were calculated: baseline concentration (Cbaseline), maximum concentration (Cmax), average concentration at steady-state (Cav,ss), time to maximum concentration (tmax), minimum concentration (Cmin), and area under the concentration-time curve from time zero to last quantifiable concentration at time t (AUC0-t). Blood was collected on Day 13 at the indicated timepoints. If plasmapheresis was performed on a dosing day, blood samples were obtained before plasmapheresis, prior to study drug administration (post-plasmapheresis), and at time points relative to the start of infusion.

Outcome measures

Outcome measures
Measure
Placebo
n=9 Participants
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 Participants
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Area Under The Concentration-Time Curve (AUC) of C1 INH Functional Activity
AUC0-48hours, n=6, 8
9.82 Units*h/mL
Standard Deviation 19.3
34.1 Units*h/mL
Standard Deviation 24.6
Area Under The Concentration-Time Curve (AUC) of C1 INH Functional Activity
AUClast, n=9, 9
30.8 Units*h/mL
Standard Deviation 50.6
113 Units*h/mL
Standard Deviation 86.7

Adverse Events

Placebo

Serious events: 3 serious events
Other events: 6 other events
Deaths: 0 deaths

CINRYZE

Serious events: 1 serious events
Other events: 9 other events
Deaths: 0 deaths

Serious adverse events

Serious adverse events
Measure
Placebo
n=9 participants at risk
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 participants at risk
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Gastrointestinal disorders
Diarrhoea
11.1%
1/9
0.00%
0/9
Immune system disorders
Transplant rejection
11.1%
1/9
0.00%
0/9
Infections and infestations
Clostridium difficile colitis
11.1%
1/9
0.00%
0/9
Injury, poisoning and procedural complications
Incisional hernia
0.00%
0/9
11.1%
1/9
Metabolism and nutrition disorders
Dehydration
11.1%
1/9
0.00%
0/9

Other adverse events

Other adverse events
Measure
Placebo
n=9 participants at risk
Participants received an intravenous (IV) infusion of normal saline, at a rate of approximately 1 mL per minute as tolerated, 7 times over a 2-week period: an initial infusion on Day 1, followed by infusions on Days 3, 5, 7, 9, 11, and 13.
CINRYZE
n=9 participants at risk
Participants received an intravenous (IV) infusion of human C1 esterase inhibitor (CINRYZE) at a rate of approximately 1 mL per minute as tolerated. Participants received a total of 7 doses over a 2-week period: an initial IV infusion of 5000 U (not to exceed 100 U/kg) on Day 1, followed by 2500 U (not to exceed 50 U/kg) IV on Days 3, 5, 7, 9, 11, and 13.
Skin and subcutaneous tissue disorders
Pruritus
0.00%
0/9
22.2%
2/9
Vascular disorders
Orthostatic hypotension
11.1%
1/9
0.00%
0/9
General disorders
Medical device complication
0.00%
0/9
11.1%
1/9
General disorders
Oedema peripheral
0.00%
0/9
33.3%
3/9
Immune system disorders
Drug hypersensitivity
0.00%
0/9
11.1%
1/9
Immune system disorders
Hypersensitivity
0.00%
0/9
11.1%
1/9
Immune system disorders
Transplant rejection
11.1%
1/9
0.00%
0/9
Infections and infestations
Cellulitis
0.00%
0/9
11.1%
1/9
Infections and infestations
Clostridium difficile colitis
11.1%
1/9
11.1%
1/9
Infections and infestations
Respiratory syncytial virus infection
11.1%
1/9
0.00%
0/9
Infections and infestations
Respiratory tract infection
0.00%
0/9
11.1%
1/9
Infections and infestations
Urinary tract infection
0.00%
0/9
22.2%
2/9
Injury, poisoning and procedural complications
Incisional hernia
0.00%
0/9
11.1%
1/9
Injury, poisoning and procedural complications
Perinephric collection
0.00%
0/9
11.1%
1/9
Injury, poisoning and procedural complications
Procedural pain
11.1%
1/9
0.00%
0/9
Injury, poisoning and procedural complications
Wound complication
0.00%
0/9
11.1%
1/9
Injury, poisoning and procedural complications
Wound secretion
0.00%
0/9
11.1%
1/9
Investigations
Hepatic enzyme increased
11.1%
1/9
0.00%
0/9
Metabolism and nutrition disorders
Decreased appetite
11.1%
1/9
0.00%
0/9
Metabolism and nutrition disorders
Dehydration
22.2%
2/9
0.00%
0/9
Metabolism and nutrition disorders
Hyperkalaemia
11.1%
1/9
11.1%
1/9
Metabolism and nutrition disorders
Hypocalcaemia
11.1%
1/9
0.00%
0/9
Metabolism and nutrition disorders
Hypokalaemia
11.1%
1/9
0.00%
0/9
Metabolism and nutrition disorders
Hypomagnesaemia
22.2%
2/9
11.1%
1/9
Metabolism and nutrition disorders
Hypophosphataemia
22.2%
2/9
11.1%
1/9
Metabolism and nutrition disorders
Obesity
0.00%
0/9
11.1%
1/9
Musculoskeletal and connective tissue disorders
Back pain
0.00%
0/9
11.1%
1/9
Nervous system disorders
Disturbance in attention
0.00%
0/9
11.1%
1/9
Psychiatric disorders
Insomnia
0.00%
0/9
11.1%
1/9
Renal and urinary disorders
Dysuria
0.00%
0/9
11.1%
1/9
Skin and subcutaneous tissue disorders
Night sweats
0.00%
0/9
11.1%
1/9
Blood and lymphatic system disorders
Anaemia
11.1%
1/9
0.00%
0/9
Blood and lymphatic system disorders
Neutropenia
11.1%
1/9
11.1%
1/9
Cardiac disorders
Tachycardia
11.1%
1/9
11.1%
1/9
Eye disorders
Conjunctivitis
0.00%
0/9
11.1%
1/9
Eye disorders
Vision blurred
0.00%
0/9
11.1%
1/9
Gastrointestinal disorders
Abdominal distension
11.1%
1/9
0.00%
0/9
Gastrointestinal disorders
Abdominal pain
11.1%
1/9
0.00%
0/9
Gastrointestinal disorders
Diarrhoea
11.1%
1/9
33.3%
3/9
Gastrointestinal disorders
Dyspepsia
0.00%
0/9
22.2%
2/9
Gastrointestinal disorders
Dysphagia
0.00%
0/9
11.1%
1/9
Gastrointestinal disorders
Erosive oesophagitis
11.1%
1/9
0.00%
0/9
Gastrointestinal disorders
Gastritis
0.00%
0/9
11.1%
1/9
Gastrointestinal disorders
Gastrooesophageal reflux disease
0.00%
0/9
11.1%
1/9
Gastrointestinal disorders
Haemorrhoidal haemorrhage
0.00%
0/9
11.1%
1/9
Gastrointestinal disorders
Mouth ulceration
0.00%
0/9
11.1%
1/9
Gastrointestinal disorders
Nausea
33.3%
3/9
11.1%
1/9
Gastrointestinal disorders
Vomiting
33.3%
3/9
0.00%
0/9
General disorders
Inflammatory pain
0.00%
0/9
11.1%
1/9

Additional Information

Study Director

Shire

Phone: +1 866 842 5335

Results disclosure agreements

  • Principal investigator is a sponsor employee If a multicenter publication is not submitted within twelve (12) months after conclusion, abandonment or termination of the Study at all sites, or after Sponsor confirms there shall be no multicenter Study publication, the Institution and/or such Principal Investigator may publish the results from the Institution site individually.
  • Publication restrictions are in place

Restriction type: OTHER