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Study to Evaluate the Clinical Efficacy and Safety of Subcutaneously Administered C1 Esterase Inhibitor for the Prevention of Angioedema Attacks in Adolescents and Adults With Hereditary Angioedema

Phase 3
Completed
Conditions
Hereditary Angioedema (HAE)
Interventions
Drug: C1 esterase inhibitor [human] liquid
Drug: Placebo
Registration Number
NCT02584959
Lead Sponsor
Shire
Brief Summary

The purpose of this study is to assess the efficacy and safety of subcutaneous administration of a liquid formulation of C1 esterase inhibitor for the prevention of angioedema attacks in adolescent and adult subjects with hereditary angioedema.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
75
Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

Study Type
INTERVENTIONAL
Study Design
CROSSOVER
Arm && Interventions
GroupInterventionDescription
Experimental/PlaceboC1 esterase inhibitor [human] liquidSubjects will be randomized to receive C1 Esterase Inhibitor in the 1st Treatment period and then switch to Placebo in the 2nd treatment period.
Experimental/PlaceboPlaceboSubjects will be randomized to receive C1 Esterase Inhibitor in the 1st Treatment period and then switch to Placebo in the 2nd treatment period.
Placebo/ExperimentalC1 esterase inhibitor [human] liquidSubjects will be randomized to receive a placebo treatment in the 1st Treatment period and then switch to receive C1 Esterase Inhibitor in the 2nd treatment period.
Placebo/ExperimentalPlaceboSubjects will be randomized to receive a placebo treatment in the 1st Treatment period and then switch to receive C1 Esterase Inhibitor in the 2nd treatment period.
Experimental/ ExperimentalC1 esterase inhibitor [human] liquidSubjects will be randomized and receive C1 Esterase Inhibitor in both 1st as well as the 2nd treatment period
Primary Outcome Measures
NameTimeMethod
Time-Normalized Number of Attacks (NNA) for Participants During a Treatment PeriodWeeks 1 to 14 for treatment period 1 and 2

The angioedema attacks were recorded in the electronic patient diary. The investigator completed a separate angioedema eCRF for each attack based on the review of the patients diary. Time-normalized number of angioedema attacks was expressed as the number of attacks per month (ie, 30.4 days) of exposure. NNA=30.4 \* (number of attacks during treatment period) / (days of treatment period).

Secondary Outcome Measures
NameTimeMethod
Time-Normalized Number of Attacks (NNA) for Participants During Each Treatment Period Excluding the First 2 Weeks.Weeks 3 to 14 for treatment period 1 and 2

The angioedema attacks were recorded in the electronic patient diary. The investigator completed a separate angioedema eCRF for each attack based on the review of the patients diary. Time-normalized number of angioedema attacks was expressed as the number of attacks per month (ie, 30.4 days) of exposure. NNA=30.4 \* (number of attacks during treatment period) / (days of treatment period).

Proportion of Participants Meeting at Least a 50% Reduction in NNA (Normalized Number of Angioedema Attacks) During the Experimental Injection Treatment Period Relative to the Pre-treatment Assessment.Weeks 1 to 14 for treatment period 1 and 2

The angioedema attacks were recorded in the electronic patient diary. The investigator completed a separate angioedema eCRF for each attack based on the review of the patients diary. Time-Normalized Number of Attacks was expressed as the number of attacks per month (ie, 30.4 days) of exposure. NNA = 30.4 x (number of attacks during treatment period) / (days of treatment period).

Cumulative Attack SeverityWeeks 1 to 14 for treatment period 1 and 2

Severity of the angioedema attack sign/symptom was characterized as None: no symptom; Mild: noticeable symptom but easily tolerated by the participant and did not interfere with routine activities; Moderate: symptom interfered with the participant's ability to attend school or participate in family life and social/recreational activities; Severe: symptom significantly limited the participant's ability to attend school or participate in family life and social/recreational activities. Symptom severity score was assigned as Mild = 1, Moderate = 2 and Severe = 3. Cumulative attack severity score was the sum of the maximum symptom severity scores recorded for each angioedema attack in a treatment period. Cumulative attack-severity score normalized per month \[(raw score/number of days of participation in that treatment period)\*30.4\] was reported here. Cumulative attack-severity score normalized per month ranged from 0 to 19.83 and higher scores represent worse symptoms.

Proportion of Participants Meeting at Least a 50% Reduction in NNA (Normalized Number of Angioedema Attacks) During the Experimental Injection Treatment Period Relative to the Placebo Period.Weeks 1 to 14 for treatment period 1 and 2

The angioedema attacks were recorded in the electronic patient diary. The investigator completed a separate angioedema eCRF for each attack based on the review of the patients diary. Time-Normalized Number of Attacks was expressed as the number of attacks per month (ie, 30.4 days) of exposure. NNA = 30.4 x (number of attacks during treatment period) / (days of treatment period).

Number of Patients With Positive Anti-C1 INH AntibodiesWeeks 1 to 14 for treatment period 1 and 2

Anti-C1 INH antibodies were measured during study time.

Proportion of Participants Meeting at Least a 50% Reduction in NNA (Normalized Number of Angioedema Attacks) During the Experimental Injection Treatment Period Relative to the Placebo Period Excluding the First 2 Weeks of Each Treatment Period.Weeks 3 to 14 for treatment period 1 and 2

The angioedema attacks were recorded in the electronic patient diary. The investigator completed a separate angioedema eCRF for each attack based on the review of the patients diary. Time-Normalized Number of Attacks was expressed as the number of attacks per month (ie, 30.4 days) of exposure. NNA = 30.4 x (number of attacks during treatment period) / (days of treatment period).

Number of Attack-free DaysWeeks 1 to 14 for treatment period 1 and 2

Attack free days were normalized per month.

Number of Angioedema Attacks Requiring Acute TreatmentWeeks 1 to 14 for treatment period 1 and 2

Angioedema attacks were normalized per month.

Response to Icatibant When Administered for an Acute AttackWeeks 1 to 14 for treatment period 1 and 2

The number of Acute Hereditary Angioedema Attacks that required Icatibant as acute therapy is presented by the number of Icatibant injections.

Number of Patients With Adverse Events (AEs)Weeks 1 to 14 for treatment period 1 and 2

Treatment-emergent adverse events (TEAE) were counted by the treatment most recently taken when the event occurred. Participants were counted once per category per treatment.

Number of Participants With Injection Site ReactionsWeeks 1 to 14 for treatment period 1 and 2

Injection site reactions (Erythema, Swelling, Cutaneous pain, Burning sensation, Itching/Pruritus, Warm sensation) were recorded on a designated eCRF page by the site personnel who monitored the local reaction for 1 hour after IP administration 5 times during each treatment period.

PK Parameters: AUC (0-96) and AUC (0-t) for Functional C1 INH Binding ActivityWithin 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2. In addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

AUC(0-96)=area under the plasma concentration-time curve from time zero to last measurable concentration; AUC(0-t)=area under the plasma concentration-time curve from time zero extrapolated to the end of the dosing interval tau, where tau is approximately 84 hours (ie, average of every 3 or 4 days) AUC(0-96) = AUC(0-tau).

Assess Disease Activity as Measured by the Angioedema Activity Score (AAS) Normalized Per MonthWeeks 1 to 14 for treatment period 1 and 2

Disease activity was measured using a 98-day Angioedema Activity Score (AAS). The AAS collects information of disease activity in the last 24 hours. The following items are assessed: experience of swelling, severity of the swelling, timing of the swelling, extent of discomfort due to the swelling, extent that the swelling caused limitations in daily life, and feelings of being disfigured by the swelling. The instrument uses a binary response option for the first item and a three-point response scale for the 5 items thereafter. The daily AAS was the sum of the AAS items per day. Total daily ASS scores range between 0 and 15 points. Higher values stand for higher disease activity. The normalized 98-day AAS per month for a participant is calculated by (the sum of daily AAS within a treatment period/the number of days that a subject has AAS records within the treatment period)\*30.4.

Participant Experience With Self-administration: How Many Visits for Confidence With Self-administrationWeek 14 for treatment period 1 and 2

The self-administration survey includes the number of visits needed for participants to be able to self-administer investigational product with confidence and all participants could self-administer without supervision. Visit 28 summarizes treatment period 1 of the experimental/experimental arm and treatment periods 1 and 2 of the experimental/placebo arm and the placebo/experimental arm. Visit 28b summarizes treatment period 2 of the experimental/experimental arm.

Participant Experience With Self-administration: Better Long-term Option and Preferred AdministrationWeek 14 for treatment period 1 and 2

The self-administration survey includes the number of visits needed for participants to be able to self-administer investigational product with confidence and all participants could self-administer without supervision. Visit 28 summarizes treatment period 1 of the experimental/experimental arm and treatment periods 1 and 2 of the experimental/placebo arm and the placebo/experimental arm. Visit 28b summarizes treatment period 2 of the experimental/experimental arm.

PK Parameters: Cmax and Cmin for Complement C4 Concentrations (Treatment C1 INH)Within 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2 and in addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

Cmax=maximum observed plasma concentration and Cmin=minimum observed plasma concentration

Mean Change in Angioedema Quality of Life Questionnaire Scores From Baseline to Week 13Baseline to week 13 for treatment period 1 and 2

The AE-QoL is a questionnaire on the quality of life of patients suffering from recurrent angioedema. It consists of 17 specific questions that are associated with work, physical activity, free time, social relations, and food. Each of the 17 questions has a five-point response scale ranging from 1 (Never) to 5 (Very Often). The AE-QoL consists of 4 dimensions (functioning=4 questions(qns) fatigue/mood=5 qns, fears/shame=6 qns, nutrition=2 qns) and a total score (all 17 questions).All scores were calculated by using the following formula: (Σ items - min Σ items / max Σ items - min Σ items) x 100. Σ items=sum of response by participant, min Σ items=minimum response possible, max Σ items=maximum response possible. Scores range from 0 to 100 , with higher scores indicating greater impairment. Absolute change calculated as visit score at week 13 minus score at baseline per period.

PK Parameters: AUC (0-96) and AUC (0-t) for C1 INH Antigen ConcentrationsWithin 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2. In addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

AUC(0-96)=area under the plasma concentration-time curve from time zero to last measurable concentration; AUC(0-t)=area under the plasma concentration-time curve from time zero extrapolated to the end of the dosing interval tau, where tau is approximately 84 hours (ie, average of every 3 or 4 days) AUC(0-96) = AUC(0-tau).

PK Parameters: AUC (0-96) and AUC (0-t) for Complement C4 Concentrations (Treamtment C1 INH)Within 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2. In addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

AUC(0-96)=area under the plasma concentration-time curve from time zero to last measurable concentration; AUC(0-t)=area under the plasma concentration-time curve from time zero extrapolated to the end of the dosing interval tau, where tau is approximately 84 hours (ie, average of every 3 or 4 days) AUC(0-96) = AUC(0-tau).

PK Parameters: AUC (0-96) and AUC (0-t) for Complement C4 Concentrations (Treatment Placebo)Within 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2. In addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

AUC(0-96)=area under the plasma concentration-time curve from time zero to last measurable concentration; AUC(0-t)=area under the plasma concentration-time curve from time zero extrapolated to the end of the dosing interval tau, where tau is approximately 84 hours (ie, average of every 3 or 4 days) AUC(0-96) = AUC(0-tau). Participant wise data was reported for this outcome.

PK Parameters: Cmax and Cmin for Functional C1 INH Binding ActivityWithin 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2 and in addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

Cmax=maximum observed plasma concentration and Cmin=minimum observed plasma concentration

PK Parameters: Cmax and Cmin for C1 INH Antigen ConcentrationsWithin 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2 and in addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2

Cmax=maximum observed plasma concentration and Cmin=minimum observed plasma concentration

PK Parameters: Cmax and Cmin for Complement C4 Concentrations (Treatment Placebo)Within 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2 and in addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

Cmax=maximum observed plasma concentration and Cmin=minimum observed plasma concentration. Participant wise data was reported for this outcome.

PK Parameters: TmaxWithin 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2 and in addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

tmax=time of maximum observed plasma concentration

PK Parameters: Tmax for Complement C4 Concentrations (Placebo Group)Within 15 min prior dosing at week 1, week 2, week 8, week 16, week 24, week 27/28 and 48 (± 3) hours after dose in week 27/28 in period 1 and 2 and in addition 24 (±3) hours, 72 (±6) hours and 96 (±6) hours post dose in week 28 for period 2.

tmax=time of maximum observed plasma concentration. Participant wise data was reported for this outcome.

Participant Experience With Self-administration: Overall Experience With the SyringeWeek 14 for treatment period 1 and 2

Self-administration survey with questions about the overall experience with the syringe was assessed in week 14 (visit 28 and 28b). Visit 28 summarizes treatment period 1 of the experimental/experimental arm and treatment periods 1 and 2 of the experimental/placebo arm and the placebo/experimental arm. Visit 28b summarizes treatment period 2 of the experimental/experimental arm.

Trial Locations

Locations (27)

AIRE Medical of Los Angeles

🇺🇸

Santa Monica, California, United States

Atlanta Allergy and Asthma Clinic

🇺🇸

Marietta, Georgia, United States

Bay Area Allergy

🇺🇸

Walnut Creek, California, United States

Asthma and Allergy Associates PC

🇺🇸

Colorado Springs, Colorado, United States

Institute For Asthma and Allergy

🇺🇸

Chevy Chase, Maryland, United States

Washington University

🇺🇸

Saint Louis, Missouri, United States

Allergy Asthma and Immunology

🇺🇸

Fair Lawn, New Jersey, United States

Clinical Research of Charlotte

🇺🇸

Charlotte, North Carolina, United States

Bernstein Clinical Research Center Inc

🇺🇸

Cincinnati, Ohio, United States

Premier Clinical Research

🇺🇸

Spokane, Washington, United States

AARA Research Center

🇺🇸

Dallas, Texas, United States

Allergy Clinic of Tulsa

🇺🇸

Tulsa, Oklahoma, United States

McMaster University Medical Center

🇨🇦

Hamilton, Ontario, Canada

Hämophilie Zentrum Rhein Main GmbH

🇩🇪

Mörfelden-Walldorf, Germany

Hospital Universitario Vall d'Hebron

🇪🇸

Barcelona, Spain

Hospital Universitari i Politecnic La Fe de Valencia

🇪🇸

Valencia, Spain

Hospital Universitario Virgen del Rocio

🇪🇸

Sevilla, Spain

Medical Research of Arizona

🇺🇸

Scottsdale, Arizona, United States

Clinical Research Solutions

🇺🇸

Middleburg Heights, Ohio, United States

Ottawa Allergy Research Corporation

🇨🇦

Ottawa, Ontario, Canada

MediQuest Clinical Research Center

🇷🇴

Targu Mures, Romania

Hospital Universitario La Paz

🇪🇸

Madrid, Spain

Chaim Sheba Medical Center

🇮🇱

Ramat-Gan, Israel

Tel Aviv Sourasky Medical Center

🇮🇱

Tel Aviv, Israel

IMMUNOe Research Centers

🇺🇸

Centennial, Colorado, United States

Allergy Treatment Center of New Jersey

🇺🇸

Iselin, New Jersey, United States

Semmelweis Egyetem

🇭🇺

Budapest, Hungary

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