Study of Sparsentan Treatment in Pediatrics With Proteinuric Glomerular Diseases

NCT ID: NCT05003986

Last Updated: 2025-12-23

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

RECRUITING

Clinical Phase

PHASE2

Total Enrollment

67 participants

Study Classification

INTERVENTIONAL

Study Start Date

2021-08-12

Study Completion Date

2027-04-12

Brief Summary

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To evaluate the safety, efficacy and tolerability of sparsentan oral suspension and tablets, and assess changes in proteinuria after once-daily dosing over 108 weeks.

Detailed Description

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This is a multicenter, open-label, 112-week study of sparsentan in approximately 67 pediatric subjects aged ≥1 year to \<18 years with selected proteinuric glomerular diseases, divided into 3 populations, defined as follows:

* Population 1: Subjects with selected proteinuric glomerular diseases associated with Focal Segmental Glomerulosclerosis (FSGS) and Minimal Change Disease (MCD) histological patterns
* Population 2: Subjects with kidney biopsy-confirmed immunoglobulin A nephropathy (IgAN), immunoglobulin A vasculitis (IgAV), or Alport syndrome (AS)
* Population 3: Subjects with kidney biopsy-confirmed IgAN

The study will evaluate long-term safety, tolerability, and efficacy with pharmacokinetic (PK) evaluations at Day 1 (Baseline), Day 2 (Visit 4), and Week 12 (Visit 9) in Population 1 and Population 2. In Population 3, PK values will be evaluated at Day 1 (Baseline) and at Weeks 4, 8, 12, 24, 36, 48, 60, 72, 84, and 96. For Population 1 and Population 2, subjects will be enrolled in 3 cohorts based on age ranges. For Population 3, subjects will be enrolled in one cohort.

Study Enrollment:

* Population 1: FSGS and/or MCD (30 subjects total)

1. Cohort 1 (6 subjects): ≥8 years to \<18 years
2. Cohort 2 (18 subjects): ≥3 years to \<8 years
3. Cohort 3 (6 subjects): ≥1 year to \<3 years
* Population 2: IgAN, IgAV, or AS (27 subjects total)

1. Cohort 1 (9 subjects): ≥8 years to \<18 years
2. Cohort 2 (12 subjects): ≥5 years to \<8 years
3. Cohort 3 (6 subjects): ≥2 years to \<5 years
* Population 3: IgAN (10 subjects total) 1. 10 subjects: ≥8 years to \<18 years

Conditions

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Focal Segmental Glomerulosclerosis Minimal Change Disease Immunoglobulin A Nephropathy IgA Vasculitis Alport Syndrome

Keywords

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Alport, AS, FSGS, IgAN, IgAV, MCD, pediatrics, peds

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Population 1: FSGS and/or MCD

Subjects with selected proteinuric glomerular diseases associated with FSGS and MCD histological patterns

Group Type EXPERIMENTAL

Sparsentan

Intervention Type DRUG

Population 1: 800 mg Sparsentan (oral suspension)

Population 2: IgAN, IgAV, or AS

Subjects with kidney biopsy-confirmed immunoglobulin A nephropathy (IgAN), immunoglobulin A vasculitis (IgAV), or Alport syndrome (AS)

Group Type EXPERIMENTAL

Sparsentan

Intervention Type DRUG

Population 2: 400 mg Sparsentan (oral suspension)

Population 3: IgAN

Subjects with kidney biopsy-confirmed IgAN

Group Type EXPERIMENTAL

Sparsentan

Intervention Type DRUG

Population 3: 400 mg Sparsentan (tablets)

Interventions

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Sparsentan

Population 1: 800 mg Sparsentan (oral suspension)

Intervention Type DRUG

Sparsentan

Population 2: 400 mg Sparsentan (oral suspension)

Intervention Type DRUG

Sparsentan

Population 3: 400 mg Sparsentan (tablets)

Intervention Type DRUG

Other Intervention Names

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RE-021 RE-021 RE-021

Eligibility Criteria

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Inclusion Criteria

A subject must meet all of the following criteria to be eligible for participation in this study:

* The subject or parent/legal guardian (as appropriate) is willing and able to provide signed informed consent/assent, and where required, the subject is willing to provide assent before any screening procedures per local requirements.
* The subject has an estimated glomerular filtration rate (eGFR) ≥30 mL/min/1.73 m2 at screening.
* The subject has a mean seated blood pressure between the 5th and 95th percentile for sex and height.


* The subject is male or female ≥1 year at screening and \<18 years of age at Day 1 (Baseline).
* The subject has a UP/C ≥1.5 g/g (170 mg/mmol) at screening AND one of the following:
* Kidney biopsy-proven FSGS or MCD histological patterns and clinical presentation consistent with primary FSGS or MCD and qualifying proteinuria at screening despite history or ongoing treatment with corticosteroids and/or other immunosuppressive disease-modifying agents.
* Documentation of a genetic mutation in a podocyte protein associated with FSGS or MCD. Subjects with a documented podocytic mutation do not require kidney biopsy.
* Kidney biopsy-proven FSGS histological pattern with medical history and clinical presentation consistent with maladaptive cause of the lesion.

Note: The kidney biopsy may have been performed at any time in the past but must include light microscopy and electron microscopy characteristics and/or immunofluorescence findings consistent with FSGS or MCD.


* The subject is male or female ≥2 years at screening and \<18 years of age at Day 1 (Baseline).
* The subject has UP/C ≥0.6 g/g (68 mg/mmol) at screening AND one of the following diagnoses:
* Kidney biopsy-confirmed IgAN, IgAV, or AS
* Diagnosis of AS by genetic testing (pathogenic X-linked Collagen, Type IV, Alpha-5 (COL4A5) mutation OR autosomal-recessive mutations in both alleles of Collagen, Type IV, Alpha-3 (COL4A3) and/or Collagen, Type IV, Alpha-4 (COL4A4) OR autosomal-dominant COL4A3 and/or COL4A4 and digenic mutations \[ie, simultaneous mutations in 2 of the COL4A3, COL4A4, and COL4A5 genes\])


* The subject is male or female ≥8 years at screening and \<18 years of age at Day 1 (Baseline).
* The subject has UP/C ≥1.0 g/g (113 mg/mmol) at screening AND has kidney biopsy-confirmed IgAN
* Subject weighs ≥40 kg
* The subject has been on ACEI and/or ARB therapy for at least 12 weeks prior to screening

Exclusion Criteria

A subject who meets any of the following will be excluded from this study:

* The subject weighs \<7.3 kg at screening.
* The subject has FSGS or MCD histological pattern secondary to viral infections, drug toxicities, or malignancies.
* The subject has immunoglobulin A (IgA) glomerular deposits not in the context of primary IgAN or IgAV (ie, secondary to another condition; eg, systemic lupus erythematosus and liver cirrhosis).
* The subject has had an acute onset or presentation of glomerular disease or a diagnostic biopsy or a relapse of glomerular disease requiring new or different class of immunosuppressive treatment (including, but not limited to, systemic corticosteroids, calcineurin inhibitors and mycophenolate mofetil, abatacept, cyclophosphamide, rituximab, ofatumumab, and ocrelizumab) within 6 months before screening.
* Subjects taking chronic immunosuppressive medications (including systemic steroids) not on a stable dose for ≥1 month before screening.
* The subject requires any of the prohibited concomitant medications as defined in the study protocol.
* The subject has undergone any organ transplantation, with the exception of corneal transplants.
* The subject has a documented history of congenital or acquired heart failure (modified Ross heart failure classification for children Class II to Class IV) and/or previous hospitalization for heart failure or unexplained dyspnea, orthopnea, paroxysmal nocturnal dyspnea, ascites, and/or peripheral edema.
* The subject has hemodynamically significant cardiac valvular disease.
* The subject has clinically significant congenital vascular disease.
* The subject has jaundice, hepatitis, or known hepatobiliary disease, or alanine aminotransferase and/or aspartate aminotransferase \>2 times the upper limit of the normal range at screening.
* The subject has a history of malignancy within the past 2 years.
* The subject has a screening hematocrit \<27% (0.27 L/L) or a hemoglobin value \<9 g/dL (90 g/L).
* The subject has a screening potassium value \>5.5 milliequivalent (mEq)/L (5.5 mmol/L).
* The subject has any abnormal clinical laboratory screening values that are considered by the Investigator to be clinically significant.
* The subject has a history of allergic response to any angiotensin II antagonist or endothelin receptor antagonist, including sparsentan, or has a hypersensitivity to any of the excipients in the study medication.
* The female subject is pregnant, plans to become pregnant during the course of the study, or is breastfeeding.
* Female subjects of childbearing potential, beginning at menarche, who do not agree to use 1 highly reliable (ie, can achieve a failure rate of \<1% per year) method of contraception from 7 days before the first dose of the study medication until 28 days after the last dose of study medication. Examples of highly reliable contraception methods include stable oral, implanted, transdermal, or injected contraceptive hormones associated with the inhibition of ovulation or an intrauterine device. One additional barrier method must also be used during vaginal sexual activity, such as a diaphragm, diaphragm with spermicide (preferred), or male partner's use of male condom or male condom with spermicide (preferred), from Day 1/Randomization until 28 days after the last dose of study medication. Female subjects of childbearing potential are defined as those who are fertile after menarche, unless permanently sterile; permanent sterilization methods include hysterectomy, bilateral salpingectomy, and bilateral oophorectomy. All female subjects of childbearing potential must have a negative serum pregnancy test result at screening (Visit 1) and a negative urine pregnancy test result, with positive results confirmed by serum, at every study visit from Day 1 (Visit 3) and after.

Note: Before menarche, pregnancy testing and contraceptive use are not required. However, subjects and their parents/legal guardians must be advised that, immediately upon menarche, subjects will be required to begin pregnancy testing and initiate contraceptive use. This requirement cannot be waived.

* The subject has participated in a study of another study medication within 28 days before screening or plans to participate in such a study during the course of this study.
* The subject has had prior exposure to sparsentan.
* The subject or parent/legal guardian (as appropriate), in the opinion of the Investigator, are unable to adhere to the requirements of the study including but not limited to, a history of noncompliance and/or any other reason that causes the Investigator to believe the subject would not be a good candidate for the study.
* For Population 3 - the subject is unable to swallow the study medication tablets whole.
Minimum Eligible Age

1 Year

Maximum Eligible Age

17 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Travere Therapeutics, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Radko Komers, MD, PhD

Role: STUDY_DIRECTOR

Travere Therapeutics, Inc.

Locations

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Cedars-Sinai Medical Center

Los Angeles, California, United States

Site Status RECRUITING

Nemours Children's Hospital

Wilmington, Delaware, United States

Site Status RECRUITING

University of Miami, Leonard M. Miller School of Medicine

Miami, Florida, United States

Site Status RECRUITING

Nicklaus Children's Hospital

Miami, Florida, United States

Site Status RECRUITING

University of Iowa, Stead Family Children's Hospital

Iowa City, Iowa, United States

Site Status WITHDRAWN

Floating Hospital for Children at Tufts Medical Center

Boston, Massachusetts, United States

Site Status WITHDRAWN

C.S. Mott Children's Hospital

Ann Arbor, Michigan, United States

Site Status RECRUITING

University of Minnesota, Masonic Children's Hospital

Minneapolis, Minnesota, United States

Site Status RECRUITING

Children's Mercy Hospitals and Clinics

Kansas City, Missouri, United States

Site Status RECRUITING

Hackensack University Medical Center

Hackensack, New Jersey, United States

Site Status RECRUITING

Jersey Shore University Medical Center

Neptune City, New Jersey, United States

Site Status RECRUITING

Cohen Children's Medical Center

New Hyde Park, New York, United States

Site Status RECRUITING

Fink Children's Ambulatory Care Center

New York, New York, United States

Site Status WITHDRAWN

University of North Carolina at Chapel Hill

Chapel Hill, North Carolina, United States

Site Status RECRUITING

Atrium Health Levine Children's Hospital

Charlotte, North Carolina, United States

Site Status COMPLETED

Duke Molecular Physiology Institute

Durham, North Carolina, United States

Site Status RECRUITING

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status RECRUITING

University of Oklahoma Health Sciences Center (OUHSC)

Oklahoma City, Oklahoma, United States

Site Status RECRUITING

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

St. Christopher's Hospital for Children

Philadelphia, Pennsylvania, United States

Site Status WITHDRAWN

Texas Children's Hospital

Houston, Texas, United States

Site Status RECRUITING

UT Health - John P. and Kathrine G. McGovern Medical School

Houston, Texas, United States

Site Status WITHDRAWN

Seattle Children's Hospital

Seattle, Washington, United States

Site Status RECRUITING

Uniklinik Köln, Klinik und Poliklinik für Kinder- und Jugendmedizin

Cologne, , Germany

Site Status RECRUITING

Universitätsklinikum Hamburg-Eppendorf

Hamburg, , Germany

Site Status RECRUITING

Universitätsklinikum Heidelberg - Angelika Lautenschläger - Kinderklinik

Heidelberg, , Germany

Site Status RECRUITING

Policlinico Bari Ospedale Pediatrico Giovanni XXIII

Bari, , Italy

Site Status RECRUITING

IRCCS Istituto Giannina Gaslini

Genova, , Italy

Site Status RECRUITING

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

Milan, , Italy

Site Status RECRUITING

Azienda Ospedale Università di Padova

Padua, , Italy

Site Status RECRUITING

Ospedale Pediatrico Bambino Gesù

Roma, , Italy

Site Status RECRUITING

Emma Kinderziekenhuis

Amsterdam, , Netherlands

Site Status RECRUITING

Radboud Universitair Medisch Centrum

Nijmegen, , Netherlands

Site Status RECRUITING

Uniwersytecki Szpital Dziecięcy w Krakowie

Krakow, , Poland

Site Status RECRUITING

Instytut Centrum Zdrowia Matki Polki

Lodz, , Poland

Site Status WITHDRAWN

Instytut Pomnik - Centrum Zdrowia Dziecka

Warsaw, , Poland

Site Status WITHDRAWN

Hospital Universitari Vall d'Hebrón

Barcelona, , Spain

Site Status RECRUITING

Hospital Universitario 12 de Octubre

Madrid, , Spain

Site Status RECRUITING

Hospital Universitario La Paz

Madrid, , Spain

Site Status RECRUITING

Hospital Universitario Virgen del Rocío

Seville, , Spain

Site Status RECRUITING

Drottning Silvias Barn- och Ungdomssjukhus

Gothenburg, , Sweden

Site Status RECRUITING

Karolinska Universitetssjukhuset Huddinge

Stockholm, , Sweden

Site Status RECRUITING

University Hospitals Bristol and Weston NHS Foundation Trust, Bristol Royal Hospital for Children

Bristol, , United Kingdom

Site Status RECRUITING

NHS Greater Glasgow and Clyde, Royal Hospital for Children

Glasgow, , United Kingdom

Site Status RECRUITING

Alder Hey Children's NHS Foundation Trust

Liverpool, , United Kingdom

Site Status RECRUITING

Great Ormond Street Hospital for Children NHS Foundation Trust

London, , United Kingdom

Site Status RECRUITING

Manchester University NHS Foundation Trust

Manchester, , United Kingdom

Site Status RECRUITING

Countries

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United States Germany Italy Netherlands Poland Spain Sweden United Kingdom

Central Contacts

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Travere Call Center

Role: CONTACT

Phone: 1-877-659-5518

Email: [email protected]

References

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Rheault MN. Treatment Approaches for Alport Syndrome. J Am Soc Nephrol. 2025 Sep 12. doi: 10.1681/ASN.0000000897. Online ahead of print.

Reference Type DERIVED
PMID: 40938675 (View on PubMed)

Related Links

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Other Identifiers

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RTRX-RE021-201

Identifier Type: -

Identifier Source: org_study_id