Efficacy and Safety of Iptacopan (LNP023) in Adult Patients With Atypical Hemolytic Uremic Syndrome Naive to Complement Inhibitor Therapy

NCT ID: NCT04889430

Last Updated: 2025-12-11

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

ACTIVE_NOT_RECRUITING

Clinical Phase

PHASE3

Total Enrollment

34 participants

Study Classification

INTERVENTIONAL

Study Start Date

2022-01-17

Study Completion Date

2029-03-29

Brief Summary

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The purpose of this Phase 3 study is to determine whether iptacopan (LNP023) is efficacious and safe for the treatment of aHUS in adult patients who are treatment naive to complement inhibitor therapy.

Detailed Description

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The study is designed as a multicenter, single-arm, open label study to demonstrate the efficacy and safety of LNP023 (iptacopan) at a dose of 200 mg b.i.d. in adult patients with aHUS who are treatment naive to complement inhibitor therapy (including anti-C5 antibody). The study will enroll approximately 50 participants and assess the effects of iptacopan on a range of efficacy assessments relevant to aHUS including hematological and kidney parameters, dialysis requirement, changes in chronic kidney disease (CKD) stage, as well as patient reported outcomes (PRO) for fatigue and quality of life.

Conditions

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Atypical Hemolytic Uremic Syndrome

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Open label single arm study

Study Groups

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Iptacopan 200 mg b.i.d

Single arm open-label with 50 adult patients receiving 200mg oral twice daily doses of iptacopan

Group Type EXPERIMENTAL

Iptacopan

Intervention Type DRUG

Iptacopan 200mg twice daily oral

Interventions

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Iptacopan

Iptacopan 200mg twice daily oral

Intervention Type DRUG

Other Intervention Names

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LNP023

Eligibility Criteria

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Inclusion Criteria

* Adult patients with evidence of active thrombotic microangiopathy (TMA), including thrombocytopenia, evidence of hemolysis, and acute kidney injury
* Vaccinations against Neisseria meningitidis, Streptococcus pneumoniae and Haemophilus influenzae infections are required prior to the start of study treatment. If the patient has not been previously vaccinated, or if a booster is required, vaccine should be given according to local regulations, at least 2 weeks prior to first study drug administration. If study treatment has to start earlier than 2 weeks post vaccination or before vaccination is given, prophylactic antibiotic treatment must be administered at the start of study treatment and for at least 2 weeks after vaccination

Exclusion Criteria

* Treatment with complement inhibitors, including anti-C5 antibody
* ADAMTS13 deficiency (\<10% activity or \<0.1U/ml), and/or Shiga toxin-related hemolytic uremic syndrome (STx-HUS), and/or Positive direct Coombs test
* Identified drug exposure-related HUS or HUS related to known genetic defects of cobalamin C metabolism or known diacylglycerol kinase ε (DGKE) mediated aHUS
* Receiving PE/PI, for 14 days or longer, prior to the start of screening for the current TMA
* Bone marrow transplantation (BMT)/hematopoietic stem cell transplantation (HSCT), heart, lung, small bowel, pancreas, or liver transplantation
* Patients with sepsis or active severe systemic bacterial, viral (including COVID-19) or fungal infection, systemic infection which confounds an accurate diagnosis of aHUS or impedes the ability to manage the aHUS disease, active infection (or history of recurrent invasive infections) caused by encapsulated bacteria
* Kidney disease suggestive of other disease than aHUS or of chronic kidney failure or family history of non-complement mediated genetic kidney disease
* Liver disease or liver injury at screening
* Systemic sclerosis (scleroderma), systemic lupus erythematosus (SLE), or antiphospholipid antibody positivity or syndrome
* Chronic hemo- or peritoneal dialysis
Minimum Eligible Age

18 Years

Maximum Eligible Age

100 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Novartis Pharmaceuticals

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Novartis Pharmaceuticals

Role: STUDY_DIRECTOR

Novartis Pharmaceuticals

Locations

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Uni Of Alabama At Birmingham

Birmingham, Alabama, United States

Site Status

USC Norris Cancer Center

Los Angeles, California, United States

Site Status

Univ of California at Los Angeles

Los Angeles, California, United States

Site Status

Univ Cali Irvine ALS Neuromuscular

Orange, California, United States

Site Status

Univ of California at Sacramento

Sacramento, California, United States

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Harbor-UCLA Medical Center .

Torrance, California, United States

Site Status

Georgetown University Lombardi Cancer Center

Washington D.C., District of Columbia, United States

Site Status

University Of Miami

Miami, Florida, United States

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Brigham and Womens Hospital

Boston, Massachusetts, United States

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University of Minnesota

Minneapolis, Minnesota, United States

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St Barnabas Hospital

Livingston, New Jersey, United States

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Rut Univ for Translational Med Scie

New Brunswick, New Jersey, United States

Site Status

Montefiore Medical Center .

The Bronx, New York, United States

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Montefiore Medical Center

The Bronx, New York, United States

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Duke University Medical Center

Durham, North Carolina, United States

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University of Cincinnati

Cincinnati, Ohio, United States

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Cleveland Clinic Foundation

Cleveland, Ohio, United States

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Comprehensive Transplant Ctr at OSU

Columbus, Ohio, United States

Site Status

UT Southwestern Medical Center

Dallas, Texas, United States

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Virginia Commonwealth University

Richmond, Virginia, United States

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Novartis Investigative Site

Vienna, , Austria

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Novartis Investigative Site

Fortaleza, Ceará, Brazil

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Novartis Investigative Site

Brasília, Federal District, Brazil

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Novartis Investigative Site

Belo Horizonte, Minas Gerais, Brazil

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Novartis Investigative Site

Recife, Pernambuco, Brazil

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Novartis Investigative Site

Porto Alegre, Rio Grande do Sul, Brazil

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Novartis Investigative Site

Botucatu, São Paulo, Brazil

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Novartis Investigative Site

São Paulo, São Paulo, Brazil

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São Paulo, São Paulo, Brazil

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São Paulo, São Paulo, Brazil

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Novartis Investigative Site

Rio de Janeiro, , Brazil

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Salvador, , Brazil

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Novartis Investigative Site

Guangzhou, Guangdong, China

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Nanjing, Jiangsu, China

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Yantai, Shandong, China

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Novartis Investigative Site

Xian, Shanxi, China

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Novartis Investigative Site

Beijing, , China

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Beijing, , China

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Beijing, , China

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Shanghai, , China

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Ostrava, Poruba, Czechia

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Novartis Investigative Site

Thiruvananthapuram, Kerala, India

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Novartis Investigative Site

Nagpur, Maharashtra, India

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Pune, Maharashtra, India

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Novartis Investigative Site

Chandigarh, Punjab, India

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Novartis Investigative Site

Chennai, Tamil Nadu, India

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Novartis Investigative Site

Vellore, Tamil Nadu, India

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Novartis Investigative Site

Hyderabad, Telangana, India

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Novartis Investigative Site

Lucknow, Uttar Pradesh, India

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Novartis Investigative Site

Iruma-gun, Saitama, Japan

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Novartis Investigative Site

Izumo, Shimane, Japan

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Novartis Investigative Site

Bunkyo Ku, Tokyo, Japan

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Novartis Investigative Site

Bratislava, Slovakia, Slovakia

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Novartis Investigative Site

Martin, Slovakia, Slovakia

Site Status

Novartis Investigative Site

Seoul, Korea, South Korea

Site Status

Novartis Investigative Site

Seoul, , South Korea

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Novartis Investigative Site

Taichung, , Taiwan

Site Status

Novartis Investigative Site

Taoyuan District, , Taiwan

Site Status

Novartis Investigative Site

Newcastle upon Tyne, Tyne and Wear, United Kingdom

Site Status

Countries

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United States Austria Brazil China Czechia India Japan Slovakia South Korea Taiwan United Kingdom

Other Identifiers

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2020-005186-13

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

CLNP023F12301

Identifier Type: -

Identifier Source: org_study_id

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