Clinical trial • Phase III • Immunology|Rare Disease

IPTACOPAN for C3 glomerulopathy

Phase III trial of IPTACOPAN for C3 glomerulopathy.

Overview

Trial Therapeutic Area
Immunology|Rare Disease
Trial Disease
C3 glomerulopathy
Trial Stage
Phase III
Drug Modality
Small molecule
Paediatric Trial
Yes
Orphan Drug
Yes

Key dates

Initial CTIS Submission Date
29-05-2024
First CTIS Authorization Date
08-07-2024

Trial design

Randomised, placebo matching iptacopan (placebo 0 mg hard gelatin capsule size 1, placebo to lnp023 100 mg; placebo 0 mg hard gelatin capsule size 0, placebo to lnp023 200 mg).-controlled Phase III trial across 22 sites in France, Germany, Greece and others.

Randomised
Yes
Comparator
Placebo matching iptacopan (Placebo 0 mg hard gelatin capsule size 1, placebo to LNP023 100 mg; Placebo 0 mg hard gelatin capsule size 0, placebo to LNP023 200 mg).
Target Sample Size
74
Trial Duration For Participant
365

Eligibility

Recruits 74 paediatric patients.

Vulnerable Population
Adolescent participants (age ≥12 and ≤17 years) are included; the trial includes provisions for assent and parental/legal guardian consent. Subject information and informed consent/assent documents are provided for adolescents (Adolescent Assent forms) and for Parent/Legal Guardian consent (Parent Legal Guardian forms). There are also 'Adolescent Becoming Adult' and specific follow-up documents (e.g., follow up for pregnant participant). Country-specific consent/assent materials exist (documents in Italian, Greek, German, French, Spanish, Dutch and English are present). Germany has an additional requirement for adolescent participants to have reached Tanner stage IV or V at screening.

Inclusion criteria

  • {"criterion_text":"- Male and female participants age ≥ 18 and ≤ 60 years (adult cohort) or age ≥ 12 and ≤ 17 years (adolescent cohort) at screening.\n- Diagnosis of C3G as confirmed by renal biopsy within 12 months prior to screening in adults and within 3 years of screening in adolescents (a biopsy report, review and confirmation by the Investigator is required). If such a biopsy is not available, confirmation may be obtained using tissue from the Day -45 biopsy (adults only) for local assessment (tissue may be processed, evaluated, and reported by Arkana Laboratories but eligibility is determined by the Investigator).\n- Prior to randomization, all participants must have been on a maximally recommended or tolerated dose of renin-angiotensin system inhibitors (RASi), e.g., an angiotensin converting enzyme inhibitor (ACEi) or an angiotensin receptor blocker (ARB) for at least 90 days. The doses of other antiproteinuric medications including mycophenolic acids, corticosteroids, and mineralocorticoid receptor antagonists should be stable for at least 90 days prior to randomization.\n- Reduced serum C3 (defined as less than 0.85 x lower limit of the central laboratory normal range) at Screening.\n- UPCR ≥ 1.0 g/g sampled from the first morning void urine sample at Day -75 and Day -15.\n- Estimated GFR (using the CKD-EPI formula for adults and modified Schwartz formula for adolescents) or measured GFR ≥ 30 ml/min/1.73m2 at Screening and Day -15.\n- Vaccination against Neisseria meningitidis and Streptococcus pneumoniae infection prior to the start of study treatment. If the patient has not been previously vaccinated, or if a booster is required, the vaccine should be given according to local guidelines at least 2 weeks prior to the first administration of study treatment. If study treatment has to start earlier than 2 weeks post vaccination, prophylactic antibiotic treatment should be initiated.\n- Vaccination against Haemophilus influenzae infections is recommended according to local guidelines, at least 2 weeks prior to the first study treatment administration.\n- In Germany only, adolescent participants must also have reached Tanner stage IV or V at the Screening visit to be enrolled in the study."}

Exclusion criteria

  • {"criterion_text":"- Participants who have received any cell or organ transplantation, including kidney transplantation.\n- Rapidly progressive crescentic glomerulonephritis defined as a 50% decline in the eGFR within 3 months with renal biopsy findings of glomerular crescent formation seen in at least 50% of glomeruli.\n- Renal biopsy showing interstitial fibrosis/tubular atrophy (IF/TA) of more than 50%.\n- Monoclonal gammopathy of undetermined significance (MGUS) confirmed by the measurement of serum free light chains or other investigation as per local standard of care.\n- Participants with an active systemic bacterial, viral or fungal infection within 14 days prior to study treatment administration or the presence of fever ≥ 38°C (100.4°F) within 7 days prior to study treatment administration.\n- A history of recurrent invasive infections caused by encapsulated organisms, e.g., meningococcus or pneumococcus.\n- The use of inhibitors of complement factors (e.g., Factor B, Factor D, C3 inhibitors, anti Complement component 5 (C5) antibodies, C5a receptor antagonists) within 6 months prior to the Screening visit.\n- The use of immunosuppressants (except mycophenolic acids), cyclophosphamide or systemic prednisone at a dose >7.5 mg/day (or equivalent for a similar medication) within 90 days of study drug administration. The use of mycophenolic acids is not permitted within 90 days prior to randomization in India.\n- Acute post-infectious glomerulonephritis at screening based upon the opinion of the investigator."}

Endpoints

Primary endpoints

  • {"endpoint_text":"- Primary endpoint for adult participants: - Log-transformed ratio to baseline in UPCR (sampled from a 24-hour urine collection) Primary endpoint for adolescent participants: - Log-transformed ratio to baseline in UPCR (sampled from a 24-hour urine collection).","definition_or_measurement_approach":"UPCR (urine protein-to-creatinine ratio) sampled from a 24-hour urine collection; endpoint is the log-transformed ratio to baseline in UPCR."}

Secondary endpoints

  • {"endpoint_text":"- Secondary endpoint for adult participants: - Change from baseline in eGFR.\n- Secondary endpoint for adult participants: - A participant meets the requirements of the composite renal endpoint if he/she satisfies: (1) a stable or improved eGFR compared to the baseline visit (≤15% reduction in eGFR), and (2) a ≥50% reduction in UPCR compared to the baseline visit.\n- Secondary endpoint for adult participants: - Change from baseline in disease total activity score in a renal biopsy.\n- Secondary endpoint for adult participants: - Change in the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT Fatigue) score.\n- Secondary endpoint for adult participants: - Occurrence of clinically significant vital signs (msDBP, msSBP, heart rate), ECGs, and safety laboratory measurements, as well as adverse events (AEs), AEs of special interest, and study drug discontinuation due to an AE.\n- Secondary endpoint for adolescent participants: - Change from baseline in eGFR.\n- Secondary endpoint for adolescent participants: - A participant meets the requirements of the composite renal endpoint if he/she satisfies the following criteria at the 6-month time point: (1) a stable or improved eGFR compared to the baseline visit (≤15% reduction in eGFR), and (2) a ≥50% reduction in UPCR compared to the baseline visit.\n- Secondary endpoint for adolescent participants: - Change from baseline to 6 months in the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue) score.\n- Secondary endpoint for adolescent participants: - Occurrence of clinically significant vital signs, ECGs, and safety laboratory measurements, as well as adverse events (AEs), AEs of special interest, and study drug discontinuation due to an AE (or any safety issue) during the double-blind period of the study.","definition_or_measurement_approach":"eGFR change from baseline (adults: CKD-EPI; adolescents: modified Schwartz formula as used in eligibility), composite renal endpoint defined as (1) stable or improved eGFR vs baseline (≤15% reduction) AND (2) ≥50% reduction in UPCR vs baseline; renal biopsy total activity score change from baseline; patient-reported fatigue measured by FACIT-Fatigue; safety endpoints include vital signs (msDBP, msSBP, heart rate), ECGs, safety laboratory measurements, adverse events, AEs of special interest, and discontinuation due to AE."}

Recruitment

Digital Remote Recruitment
Yes
Planned Sample Size
74
Recruitment Window Months
66
Consent Approach
Adults provide written informed consent using country-specific Main ICF documents. Adolescents (age 12–17) provide assent using 'Adolescent Assent' forms while Parent/Legal Guardian consent is required (Parent Legal Guardian forms are provided). There are also 'Adolescent Becoming Adult' and specific follow-up consent documents (including pregnancy follow-up). Consent/assent materials are available in multiple languages (English and country languages shown in the submission: Italian, Greek, German, French, Spanish, Dutch, etc.). Separate data protection consent and optional assessment consents are provided where applicable.

Methods

  • Country-specific recruitment advertisements/documents (K2_Advertisements / K1_Recruitment Arrangements) present for multiple countries (documents labeled for Italy, Germany, Greece, France, Netherlands, etc.).
  • Use of third-party vendors for patient recruitment and retention and patient engagement: Greenphire LLC (Patient Recruitment & Retention - patient travel and accommodation), Myonex LLC (Patient Recruitment & Retention Materials), Publicis Healthcare Communications Group Limited (Patient Recruitment & Retention Material: Participant Safety Card, Participant Workbook & Study Logo), and other vendors supporting patient recruitment/engagement (e.g., Jumo Health USA Inc. - patient education and engagement).
  • Provision of ePRO materials and translations (Kayentis, RWS Life Sciences, IQVIA-related services) to support patient-reported outcomes collection and participant communications.

Geography

Total Number Of Sites
22
Total Number Of Participants
37

France

Latest Decision Or Authorization Date
17-02-2026
Number Of Sites
7
Number Of Participants
7

Sites

Site Name
Centre Hospitalier Universitaire De Lille
Department Name
#3205: Service de néphrologie pédiatrique
Principal Investigator Name
François Provot
Principal Investigator Email
provotf@gmail.com
Contact Person Name
François Provot
Contact Person Email
provotf@gmail.com
Site Name
Assistance Publique Hopitaux De Paris
Department Name
#3206: - Service de Néphrologie et Transplantation Adulte - Service de Néphrologie Pédiatrique
Principal Investigator Name
Aude Servais
Principal Investigator Email
aude.servais@nck.aphp.fr
Contact Person Name
Aude Servais
Contact Person Email
aude.servais@nck.aphp.fr
Site Name
Assistance Publique Hopitaux De Paris
Department Name
#3207: Département de Néphrologie Pédiatrique
Principal Investigator Name
Julien Hogan
Principal Investigator Email
Julien.hogan2@aphp.fr
Contact Person Name
Julien Hogan
Contact Person Email
Julien.hogan2@aphp.fr
Site Name
Assistance Publique Hopitaux De Paris
Department Name
#3202: Service de Néphrologie et de dialyse
Principal Investigator Name
Sophie Chauvet
Principal Investigator Email
sophie.chauvet@aphp.fr
Contact Person Name
Sophie Chauvet
Contact Person Email
sophie.chauvet@aphp.fr
Site Name
Centre Hospitalier Universitaire De Montpellier
Department Name
#3201: Département: Néphrologie/Transplantation
Principal Investigator Name
Moglie Le Quintrec-Donnette
Principal Investigator Email
m-lequintrec-donnette@chu-montpellier.fr
Contact Person Name
Moglie Le Quintrec-Donnette
Site Name
Centre Hospitalier Universitaire De Lille
Department Name
#3205: Service de Néphrologie Adulte
Principal Investigator Name
François Provot
Principal Investigator Email
provotf@gmail.com
Contact Person Name
François Provot
Contact Person Email
provotf@gmail.com
Site Name
Centre Hospitalier Universitaire De Lille
Department Name
Avenue Eugene Avinee (site address listed)
Principal Investigator Name
François Provot
Principal Investigator Email
provotf@gmail.com
Contact Person Name
François Provot
Contact Person Email
provotf@gmail.com

Germany

Latest Decision Or Authorization Date
13-05-2026
Number Of Sites
2
Number Of Participants
8

Sites

Site Name
Universitaetsklinikum Heidelberg AöR
Department Name
#3311: Klinik Kinderheilkunde I
Principal Investigator Name
Christian Patry
Principal Investigator Email
Christian.Patry@med.uni-heidelberg.de
Contact Person Name
Christian Patry
Site Name
University Hospital Cologne AöR
Department Name
#3312 : Klinik und Poliklinik für Kinder- und Jugendmedizin Pädiatrische Nephrologie
Principal Investigator Name
Sandra Habbig
Principal Investigator Email
sandra.habbig@uk-koeln.de
Contact Person Name
Sandra Habbig
Contact Person Email
sandra.habbig@uk-koeln.de

Greece

Latest Decision Or Authorization Date
13-02-2026
Number Of Sites
3
Number Of Participants
2

Sites

Site Name
Laiko General Hospital Of Athens
Department Name
3401: Kidney Transplant And Nephrology Clinic
Principal Investigator Name
Ioannis Boletis
Principal Investigator Email
laikneph@laiko.gr
Contact Person Name
Ioannis Boletis
Contact Person Email
laikneph@laiko.gr
Site Name
University General Hospital Of Heraklion
Department Name
3403: Nephrology Department
Principal Investigator Name
Konstantinos Stylianou
Principal Investigator Email
kstylianu@gmail.com
Contact Person Name
Konstantinos Stylianou
Contact Person Email
kstylianu@gmail.com
Site Name
Ippokratio General Hospital Of Thessaloniki
Department Name
3404: 1st dpt. of Pediatrics
Principal Investigator Name
Stella Stabouli
Principal Investigator Email
sstaboul@auth.gr
Contact Person Name
Stella Stabouli
Contact Person Email
sstaboul@auth.gr

Italy

Latest Decision Or Authorization Date
23-03-2026
Number Of Sites
3
Number Of Participants
11

Sites

Site Name
Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico
Department Name
3603: S.C. Nefrologia e Dialisi Pediatrica Trapianti di Rene Padiglione De Marchi
Principal Investigator Name
Gianluigi Ardissino
Principal Investigator Email
ardissino@centroseu.org
Contact Person Name
Gianluigi Ardissino
Contact Person Email
ardissino@centroseu.org
Site Name
Bambino Gesu Childrens Hospital
Department Name
3602: U.O. Nefrologia e Dialisi
Principal Investigator Name
Marina Vivarelli
Principal Investigator Email
marina.vivarelli@opbg.net
Contact Person Name
Marina Vivarelli
Contact Person Email
marina.vivarelli@opbg.net
Site Name
Istituto Di Ricerche Farmacologiche Mario Negri
Department Name
3601: Centro Ricerche Cliniche per Malattie Rare Aldo e Cele Daccò
Principal Investigator Name
Giuseppe Remuzzi
Principal Investigator Email
giuseppe.remuzzi@marionegri.it
Contact Person Name
Giuseppe Remuzzi
Contact Person Email
giuseppe.remuzzi@marionegri.it

Spain

Latest Decision Or Authorization Date
24-02-2026
Number Of Sites
4
Number Of Participants
4

Sites

Site Name
Hospital Universitari Vall D Hebron
Department Name
#3807: Nefrología pediátrica
Principal Investigator Name
Gema Ariceta Iraola
Principal Investigator Email
gema.ariceta@vallhebron.cat
Contact Person Name
Gema Ariceta Iraola
Contact Person Email
gema.ariceta@vallhebron.cat
Site Name
Hospital Universitario 12 De Octubre
Department Name
#3801: Nefrología
Principal Investigator Name
Teresa Cavero Escribano
Principal Investigator Email
tcaveroescribano@gmail.com
Contact Person Name
Teresa Cavero Escribano
Contact Person Email
tcaveroescribano@gmail.com
Site Name
Hospital Universitario Virgen De La Victoria
Department Name
#3805: Nefrología
Principal Investigator Name
Monica Martin Velazquez
Principal Investigator Email
monica.martin.velazquez@gmail.com
Contact Person Name
Monica Martin Velazquez
Site Name
Hospital Universitario Virgen De La Macarena
Department Name
#3804: Nefrología
Principal Investigator Name
Mercedes Salgueira Lazo
Contact Person Name
Mercedes Salgueira Lazo

Netherlands

Latest Decision Or Authorization Date
16-02-2026
Number Of Sites
2
Number Of Participants
4

Sites

Site Name
Leids Universitair Medisch Centrum (LUMC)
Department Name
3701: Nephrology
Principal Investigator Name
Aiko De Vries
Principal Investigator Email
a.p.j.de_vries@lumc.nl
Contact Person Name
Aiko De Vries
Contact Person Email
a.p.j.de_vries@lumc.nl
Site Name
Radboud universitair medisch centrum / RADBOUDUMC
Department Name
3702: Paediatrics
Principal Investigator Name
Nicole van de Kar
Principal Investigator Email
nicole.vandekar@radboudumc.nl
Contact Person Name
Nicole van de Kar
Contact Person Email
nicole.vandekar@radboudumc.nl

Sponsor

Primary sponsor

Full Name
Novartis Pharma AG
Organisation Type
Pharmaceutical company
Country Of Registered Address
Switzerland

Contract research organisations

Name
Parexel International (IRL) Limited
Responsibilities
Regulatory and trial support (duty code 12; clinical trial support)
Name
Icon Clinical Research Limited
Responsibilities
DMC support and clinical trial management support
Name
Syneos Health Inc.
Responsibilities
Clinical trial support (operational duties)
Name
IQVIA Limited
Responsibilities
Operational support including cardiac monitoring devices and other trial services

Third parties

  • {"country":"United States","full_name":"Bioclinica Inc.","duties_or_roles":"Imaging Assessment","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"United States","full_name":"RWS Life Sciences Inc.","duties_or_roles":"ePRO Translations","organisation_type":"Pharmaceutical company"}
  • {"country":"Greece","full_name":"IQVIA RDS Hellas Single Member S.A.","duties_or_roles":"unspecified (product role code 1 listed)","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"Q Squared Solutions Limited","duties_or_roles":"unspecified (product role code 4 listed)","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"France","full_name":"Kayentis","duties_or_roles":"Providing materials for ePROs to sites, ePRO data transfer","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"Greenphire LLC","duties_or_roles":"Patient Recruitment & Retention (Patient travel and accommodation)","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"Myonex LLC","duties_or_roles":"Patient Recruitment & Retention Materials (Cooler Bags & Tote Bags)","organisation_type":"Hospital/Clinic/Other health care facility"}
  • {"country":"United Kingdom","full_name":"Adelphi Values Limited","duties_or_roles":"CCI","organisation_type":"Pharmaceutical company"}
  • {"country":"Ireland","full_name":"Icon Clinical Research Limited","duties_or_roles":"DMC support and other trial support","organisation_type":"Pharmaceutical company"}
  • {"country":"Netherlands","full_name":"Eurofins Central Laboratory B.V.","duties_or_roles":"Central laboratory / investigator services","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Medidata Solutions Inc.","duties_or_roles":"Data capture / Rave/ EDC (product role codes 6 and 7)","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"Jumo Health USA Inc.","duties_or_roles":"Patient education and engagement","organisation_type":"Hospital/Clinic/Other health care facility"}
  • {"country":"United States","full_name":"Syneos Health Inc.","duties_or_roles":"Clinical trial support (product role code 1)","organisation_type":"Pharmaceutical company"}
  • {"country":"India","full_name":"Veeda Clinical Research Limited","duties_or_roles":"Local clinical research support","organisation_type":"Pharmaceutical company"}
  • {"country":"France","full_name":"SGS France","duties_or_roles":"Local support / services","organisation_type":"Pharmaceutical company"}
  • {"country":"Ireland","full_name":"Parexel International (IRL) Limited","duties_or_roles":"Regulatory / trial support (code 12 duties listed)","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"IQVIA Limited","duties_or_roles":"Multiple trial support roles including cardiac monitoring devices and other services","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"Publicis Healthcare Communications Group Limited","duties_or_roles":"Patient Recruitment & Retention Material (Participant Safety Card, Participant Workbook & Study Logo)","organisation_type":"Non-Pharmaceutical company"}

Investigational products

Investigational Product Name
IPTACOPAN
Active Substance
IPTACOPAN
Modality
Small molecule
Routes Of Administration
ORAL
Route
ORAL
Authorisation Status
euMpNumber PRD10338043 (product record present in submission)
Orphan Designation
Yes
Maximum Dose
400 mg
Investigational Product Name
IPTACOPAN HYDROCHLORIDE
Active Substance
IPTACOPAN HYDROCHLORIDE
Modality
Small molecule
Routes Of Administration
ORAL
Route
ORAL
Authorisation Status
euMpNumber PRD5330958 (product record present in submission)
Orphan Designation
Yes
Maximum Dose
400 mg
Investigational Product Name
Placebo 0 mg hard gelatin capsule size 1 (placebo to LNP023 100 mg)
Modality
Other
Starting Dose
0 mg (placebo)
Investigational Product Name
Placebo 0 mg hard gelatin capsule size 0 (placebo to LNP023 200 mg)
Modality
Other
Starting Dose
0 mg (placebo)

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