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A Study of Long-Term Safety and Efficacy of Lanadelumab for Prevention of Acute Attacks of Non-histaminergic Angioedema With Normal C1-Inhibitor

Phase 3
Completed
Conditions
Angioedema
Interventions
Registration Number
NCT04444895
Lead Sponsor
Shire
Brief Summary

The purpose of this study is to evaluate the long-term safety and efficacy of repeated subcutaneous (SC) administration of lanadelumab in adolescents and adults with non-histaminergic angioedema with normal C1-inhibitor who completed study SHP643-303 (NCT04206605).

Detailed Description

This study consists of 26-week treatment period (Day 0 to Day 182) and a 2-week follow-up period. Participants who completed the double-blind treatment period at Day 182 of Study SHP643-303 (NCT04206605) will enroll into this extension study.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
73
Inclusion Criteria
  • Males and females, 12 years of age and older diagnosed with non-histaminergic normal C1-INH angioedema at the time of enrollment into the antecedent Study SHP643-303 (NCT04206605).
  • Participants must have completed the treatment period (through Visit 26/Day 182) of Study SHP643-303 (NCT04206605) without reporting a clinically significant TEAE that would preclude subsequent exposure to lanadelumab.
  • Agree to adhere to the protocol-defined schedule of treatments, assessments, and procedures.
  • Males, or non-pregnant, non-lactating females who are of child-bearing potential and who agree to be abstinent or agree to comply with the applicable contraceptive requirements of this protocol for the duration of the study; or females of non-childbearing potential, defined as surgically sterile (status post hysterectomy, bilateral oophorectomy, or bilateral tubal ligation) or post-menopausal for at least 12 months.
  • The participant (or the participant's parent/legal guardian, if applicable) has provided written informed consent approved by the institutional review board/research ethics board/ethics committee (IRB/REB/EC) at any time prior to study start. If the participant is a minor (i.e. lesser then (<) 18 years of age), have a parent/legal guardian who is informed of the nature of the study provide written informed consent (i.e. permission) for the minor to participate in the study before any study-specific procedures are performed. Assent will be obtained from minor participants.
Exclusion Criteria
  • Discontinued from Study SHP643-303 (NCT04206605) after enrollment but before Visit 26 for any reason.
  • Presence of important safety concerns identified in Study SHP643-303 (NCT04206605) that would preclude participation in this study.
  • Dosing with an investigational product (IP, not including IP defined in antecedent Study SHP643-303 [NCT04206605]) or exposure to an investigational device within 4 weeks prior to Day 0.
  • Participants has a known hypersensitivity to the investigational product or its components.
  • Have any condition (surgical or medical) that, in the opinion of the investigator or sponsor, may compromise their safety or compliance, preclude the successful conduct of the study, or interfere with interpretation of the results (e.g. significant pre-existing illness or other major comorbidities that the investigator considers may confound the interpretation of study results).

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Lanadelumab 300 mg Every 2 WeeksLanadelumabParticipants received 300 milligrams (mg) lanadelumab subcutaneous (SC) injection, every 2 weeks (Q2W) for up to 26 weeks with an option to switch to lanadelumab 300 mg every 4 weeks (Q4W) if attacks were well-controlled based on the investigator's discretion and consultation with the sponsor's medical monitor.
Primary Outcome Measures
NameTimeMethod
Number of Participants With Treatment Emergent Adverse Events (TEAEs) Including Adverse Events of Special Interest (AESI) and Serious Adverse Events (SAEs) During Treatment PeriodFrom Day 0 up to Day 182

TEAE: Any event emerging or manifesting at or after initiation of treatment with investigational product (IP) or medicinal product or any existing event that worsens in either intensity or frequency following exposure to IP or medicinal product including clinically meaningful findings in laboratory safety tests, vital signs, weight, and electrocardiogram (ECG) findings. SAE: Any untoward clinical manifestation of signs, symptoms or outcomes (whether considered related to IP or not and at any dose: results in death, is life-threatening, requires inpatient hospitalization or prolongation of hospitalization, results in persistent or significant disability/incapacity, congenital abnormality/birth defect, an important medical event. AESI included hypersensitivity reactions, events of disordered coagulation such as bleeding AESI, hypercoagulable AESI. TEAEs were classified and reported as angioedema attack and non-angioedema attack adverse events in this outcome measure.

Number of Participants With Treatment Emergent Adverse Events (TEAEs) Including Adverse Events of Special Interest (AESI) and Serious Adverse Events (SAEs) During Follow-upFrom Day 183 up to Day 196

TEAE: Any event emerging or manifesting at or after initiation of treatment with investigational product (IP) or medicinal product or any existing event that worsens in either intensity or frequency following exposure to IP or medicinal product including clinically meaningful findings in laboratory safety tests, vital signs, weight, and electrocardiogram (ECG) findings. SAE: Any untoward clinical manifestation of signs, symptoms or outcomes (whether considered related to IP or not and at any dose: results in death, is life-threatening, requires inpatient hospitalization or prolongation of hospitalization, results in persistent or significant disability/incapacity, congenital abnormality/birth defect, an important medical event. AESI included hypersensitivity reactions, events of disordered coagulation such as bleeding AESI, hypercoagulable AESI. TEAEs were classified and reported as angioedema attack and non-angioedema attack adverse events in this outcome measure.

Secondary Outcome Measures
NameTimeMethod
Number of Moderate or Severe Angioedema Attacks During the Treatment Period of Day 0 Through Day 182 in Participants Who Switched Dosing RegimenFrom Day 0 up to Day 182

The overall severity of angioedema attack was determined by the site using following definitions: mild (transient or mild discomfort), moderate (mild to moderate limitation in activity), severe (marked limitation in activity). Number of moderate or severe angioedema attacks during the treatment period of Day 0 through Day 182 was assessed.

Number of High-Morbidity Angioedema Attacks During the Treatment Period of Day 0 Through Day 182From Day 0 up to Day 182

A high-morbidity angioedema attack was defined as any attack that has at least one of the following characteristics: severe, results in hospitalization (except hospitalization for observation \<24 hours), hemodynamically significant (systolic blood pressure (BP) \<90 millimetres of mercury (mmHg), requires intravenous hydration, or associated with syncope or near-syncope) or laryngeal.

Pharmacokinetic (PK) Plasma Concentrations of LanadelumabPredose on Days 0, 84, and 140 and postdose on Day 182

The data was partitioned by dosing regimen and reported accordingly to appropriately attribute to each dosing regimen.

Number of Moderate or Severe Angioedema Attacks During the Treatment Period of Day 0 Through Day 182From Day 0 up to Day 182

The overall severity of angioedema attack was determined by the site using following definitions: mild (transient or mild discomfort), moderate (mild to moderate limitation in activity), severe (marked limitation in activity). Number of moderate or severe angioedema attacks during the treatment period of Day 0 through Day 182 was assessed.

Change From Baseline in Total Angioedema Quality of Life (AE-QoL) Questionnaire Total Score at End of Treatment PeriodBaseline (Day 0) up to end of treatment period (Day 182)

The AE-QoL questionnaire is self-administered validated instrument to assess health related (HR)QoL among participants with recurrent angioedema(including hereditary angioedema\[HAE\]). It consists of 17 disease-specific QOL items, to produce total AE-QoL score \& 4 domain scores(functioning,fatigue/mood,fear/shame,nutrition) each of 17 items had 5-point response scale ranging from 1(Never) to 5(Very Often). It was scored according to developers' guidelines to produce 4 domain scores yielding total score. The raw total score(mean of all item scores) was rescaled using linear transformations into final percentage scores ranging 0-100, based on maximum possible score, where higher score, greater QoL impairment. Negative change from Baseline indicates better QoL. Baseline: Last non-missing value prior to first exposure to study drug(based on date or date/time). As pre-specified in SAP, data for this outcome measure was collected and analyzed as single group irrespective of dosing regimen.

Number of Investigator-Confirmed Angioedema Attacks During the Treatment Period of Day 0 Through Day 182 in Participants Who Switched Dosing RegimenFrom Day 0 up to Day 182

An angioedema attack was defined as the symptoms or signs consistent with an attack in at least 1 of the following locations: peripheral angioedema (cutaneous swelling involving an extremity, the face, neck, torso, and/or genitourinary region), abdominal angioedema (abdominal pain, with or without abdominal distention, nausea, vomiting, or diarrhea), laryngeal angioedema (stridor, dyspnea, difficulty speaking, difficulty swallowing, throat tightening, or swelling of the tongue, palate, uvula, or larynx). Number of investigator-confirmed angioedema attacks during the treatment period of Day 0 through Day 182 was assessed.

Number of Investigator-Confirmed Angioedema Attacks During the Treatment Period of Day 0 Through Day 182From Day 0 up to Day 182

An angioedema attack was defined as the symptoms or signs consistent with an attack in at least 1 of the following locations: peripheral angioedema (cutaneous swelling involving an extremity, the face, neck, torso, and/or genitourinary region), abdominal angioedema (abdominal pain, with or without abdominal distention, nausea, vomiting, or diarrhea), laryngeal angioedema (stridor, dyspnea, difficulty speaking, difficulty swallowing, throat tightening, or swelling of the tongue, palate, uvula, or larynx). Number of investigator-confirmed angioedema attacks during the treatment period of Day 0 through Day 182 was assessed.

Plasma Kallikrein (pKal) ActivityPredose on Days 0, 84, and 140 and postdose on Day 182

Plasma kallikrein activity was measured by biomarker cleaved high molecular weight kininogen (cHMWK) with factor XIIa activation level to assess the pharmacodynamics of lanadelumab. The data was partitioned by dosing regimen and reported accordingly to appropriately attribute to each dosing regimen.

Number of Participants With Neutralizing Antidrug Antibodies (ADA) in PlasmaPredose on Days 0, 84, and 140 and postdose on Day 182

Number of participants with positive ADA including evaluation of neutralizing antibodies in plasma was assessed. As pre-specified in the statistical analysis plan (SAP), data for this outcome measure was collected and analyzed as a single group irrespective of dosing regimen.

Number of Participants With Any Pause During InjectionDays 0, 14, 28, 42, 56, 70, 84, 98, 112, 126, 140, 154, and 168

An injection report was completed by the participant (or parent/caregiver) following each dose administration of lanadelumab injection used during the treatment period and any kind of pause during injection was captured. Categories with at least one participant with event are reported.

Number of Participants With Treatment Emergent Adverse Events (TEAEs) Including Adverse Events of Special Interest (AESI) and Serious Adverse Events (SAEs) in Participants Who Switched Dosing RegimenFrom Day 0 up to Day 196

TEAE: Any event emerging or manifesting at or after initiation of treatment with investigational product (IP) or medicinal product or any existing event that worsens in either intensity or frequency following exposure to IP or medicinal product including clinically meaningful findings in laboratory safety tests, vital signs, weight, and electrocardiogram (ECG) findings. SAE: Any untoward clinical manifestation of signs, symptoms or outcomes (whether considered related to IP or not and at any dose: results in death, is life-threatening, requires inpatient hospitalization or prolongation of hospitalization, results in persistent or significant disability/incapacity, congenital abnormality/birth defect, an important medical event. AESI included hypersensitivity reactions, events of disordered coagulation such as bleeding AESI, hypercoagulable AESI. TEAEs were classified and reported as angioedema attack and non-angioedema attack adverse events in this outcome measure.

Number of High-Morbidity Angioedema Attacks During the Treatment Period of Day 0 Through Day 182 in Participants Who Switched Dosing RegimenFrom Day 0 up to Day 182

A high-morbidity angioedema attack was defined as any attack that has at least one of the following characteristics: severe, results in hospitalization (except hospitalization for observation \<24 hours), hemodynamically significant (systolic BP \<90 mmHg, requires intravenous hydration, or associated with syncope or near-syncope) or laryngeal.

Trial Locations

Locations (35)

Optimed Research, LTD

🇺🇸

Columbus, Ohio, United States

Semmelweis Egyetem

🇭🇺

Budapest, Hungary

Universitair Medisch Centrum Groningen

🇳🇱

Groningen, Netherlands

A.O. Ospedali riuniti Villa Sofia - Cervello,

🇮🇹

Palermo, Palermo Palermo, Italy

Azienda Socio Sanitaria Territoriale Fatebenefratelli (Presidio Ospedale Sacco)

🇮🇹

Milano, Italy

Clinical Research Center of Alabama

🇺🇸

Birmingham, Alabama, United States

UCSD Angioedema Center

🇺🇸

San Diego, California, United States

Rush University Medical Center

🇺🇸

Chicago, Illinois, United States

Massachusetts General Hospital

🇺🇸

Boston, Massachusetts, United States

Bernstein Clinical Research Center, LLC

🇺🇸

Cincinnati, Ohio, United States

Seattle Allergy & Asthma Research Institute

🇺🇸

Seattle, Washington, United States

Mayo Clinic

🇺🇸

Rochester, Minnesota, United States

NZOZ Homeo Medicus, Poradnia Alergologiczna

🇵🇱

Bialystok, Poland

Asthma and Allergy Associates, PC

🇺🇸

Colorado Springs, Colorado, United States

Medical Research of Arizona

🇺🇸

Scottsdale, Arizona, United States

Allergy and Asthma Clinical Research Inc

🇺🇸

Walnut Creek, California, United States

University of Michigan

🇺🇸

Ann Arbor, Michigan, United States

Kanarek Allergy, Asthma and Immunology

🇺🇸

Overland Park, Kansas, United States

Institute for Asthma & Allergy, P.C.

🇺🇸

Chevy Chase, Maryland, United States

Washington University

🇺🇸

Saint Louis, Missouri, United States

Ottawa Allergy Research Corporation

🇨🇦

Ottawa, Ontario, Canada

Hôpital Saint-Antoine

🇫🇷

Paris, France

Clinique Specialisee en Allergie de la Capitale

🇨🇦

Québec, Quebec, Canada

Klinikum rechts der Isar der TU

🇩🇪

Muenchen, Bayern, Germany

Klinikum der Johann Wolfgang Goethe-Universitaet pt

🇩🇪

Frankfurt, Hessen, Germany

Hiroshima University Hospital

🇯🇵

Hiroshima-shi, Hiroshima-Ken, Japan

Clover Hospital

🇯🇵

Fujisawa-shi, Kanagawa-Ken, Japan

Azienda Ospedaliera Universitaria "Federico II"

🇮🇹

Napoli, Italy

Azienda Ospedaliera Universitaria OO. RR. S. Giovanni di Dio e Ruggi D'Aragona

🇮🇹

Salerno, Italy

Kobe University Hospital

🇯🇵

Kobe-shi, Hyogo-Ken, Japan

Amsterdam UMC

🇳🇱

Amsterdam, Netherlands

UMC Utrecht

🇳🇱

Utrecht, Netherlands

"ALL-MED" Specjalistyczna Opieka Medyczna Filia

🇵🇱

Wroclaw, Poland

Hospital Universitario Cruces

🇪🇸

Barakaldo, Vizcaya, Spain

Hospital Universitari Vall d'Hebron

🇪🇸

Barcelona, Spain

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