What side effects are associated with this type of intervention?

Common treatments for Henoch-Schonlein Purpura (HSP) include corticosteroids and immunosuppressants, which can cause side effects such as weight gain, hypertension, hyperglycemia, increased risk of infections, liver toxicity, and gastrointestinal disturbances. Treatments similar to Sparsentan, a dual endothelin receptor and angiotensin II receptor antagonist, may have side effects like hypotension, dizziness, and renal impairment.

What prior FDA approvals exist?

There are no specific FDA-approved treatments for Henoch-Schonlein Purpura (HSP). However, treatments often focus on managing symptoms and may include corticosteroids and immunosuppressants. Sparsentan, a dual endothelin receptor and angiotensin II receptor antagonist, is being studied for proteinuric glomerular diseases, which could have implications for conditions like HSP that involve kidney complications. Similar drugs that target the renin-angiotensin system, such as ACE inhibitors and angiotensin II receptor blockers, are commonly used to manage proteinuria in various kidney diseases.

What other types of research exist?

Promising alternatives to Sparsentan for Henoch-Schonlein Purpura patients may include other endothelin receptor antagonists, angiotensin II receptor blockers (ARBs), or novel therapies such as tolvaptan, which has shown efficacy in other proteinuric conditions. Additionally, therapies targeting inflammation and coagulation pathways, like intravenous recombinant thrombomodulin, may also be considered, although their efficacy in HSP specifically would need further investigation.

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Angiotensin Receptor Blocker and Endothelin Receptor Antagonist

Sparsentan for Proteinuric Kidney Disease (EPPIK Trial)

Q: What is the mechanism of action of this treatment?

Henoch-Schonlein Purpura (HSP) treatments often aim to manage symptoms and prevent renal complications. Sparsentan, a dual endothelin receptor and angiotensin II receptor antagonist, works by blocking endothelin-1 and angiotensin II, which are involved in vasoconstriction, inflammation, and fibrosis. This dual inhibition can reduce proteinuria and protect kidney function, which is vital for HSP patients at risk of chronic kidney disease.

Phase 2

Recruiting

Research Sponsored by Travere Therapeutics, Inc.

Eligibility Criteria Checklist

Specific guidelines that determine who can or cannot participate in a clinical trial

Must have

For Population 1: The subject is male or female ≥1 year at screening and <18 years of age at Day 1 (Baseline)

For Population 1: The subject has a documented kidney biopsy-proven FSGS histological pattern with specific clinical presentation

Must not have

The subject has FSGS or MCD histological pattern secondary to specific causes

The subject weighs <7.3 kg at screening

Timeline

Screening 3 weeks

Treatment Varies

Follow Up at scheduled day 1, day 2 and week 12 visits for population 1 and 2. at scheduled day 1 and week 4, 8, 12, 24, 36, 48, 60, 72, 84, and 96 visits for population 3.

Awards & highlights

Summary

This trial is testing sparsentan, a daily oral medication, in children with specific kidney diseases that cause high protein levels in urine. The goal is to see if it is safe and effective. Sparsentan helps by reducing protein leakage into the urine, which can protect the kidneys.

Who is the study for?

This trial is for kids and teens with certain kidney diseases like FSGS or MCD, who have high levels of protein in their urine. They must be healthy enough in other ways (like liver function and blood pressure) to take part. Kids under a specific weight or those with certain medical conditions can't join.

What is being tested?

The trial tests Sparsentan, an oral medication given once daily to see if it's safe, works well, and is tolerable over a period of 108 weeks. It specifically looks at how the drug affects the amount of protein in the urine among young patients.

What are the potential side effects?

While not explicitly listed here, potential side effects may include issues related to blood pressure changes due to Sparsentan's action on blood vessels and any common reactions from taking new medications orally.

Eligibility Criteria

Inclusion Criteria

You may be eligible if you check “Yes” for the criteria below

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I am under 18 years old and older than 1 year.

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I have FSGS confirmed by a kidney biopsy.

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My kidney biopsy shows I have FSGS or MCD.

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I have a genetic mutation linked to my kidney condition.

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My kidney function test shows a filtration rate of at least 30 mL/min.

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I am between 2 and 17 years old.

Exclusion Criteria

You may be eligible for the trial if you check “No” for criteria below:

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My kidney disease is due to FSGS or MCD.

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I weigh less than 7.3 kg.

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I have had cancer in the last 2 years.

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I needed a new type of immune system treatment for my kidney condition in the last 6 months.

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I am pregnant, planning to become pregnant, or am breastfeeding.

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I have had an organ transplant, but not of the cornea.

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I have a documented history of heart conditions.

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I have liver conditions or abnormal liver tests.

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I do not need any medication that the study does not allow.

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I have previously taken sparsentan.

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I have IgA deposits in my kidneys not due to primary IgAN or IgAV.

Timeline

Screening ~ 3 weeks3 visits

Treatment ~ Varies

Follow Up ~ at scheduled day 1, day 2 and week 12 visits for population 1 and 2. at scheduled day 1 and week 4, 8, 12, 24, 36, 48, 60, 72, 84, and 96 visits for population 3.

Screening ~ 3 weeks

Treatment ~ Varies

Follow Up ~at scheduled day 1, day 2 and week 12 visits for population 1 and 2. at scheduled day 1 and week 4, 8, 12, 24, 36, 48, 60, 72, 84, and 96 visits for population 3.

This trial's timeline: 3 weeks for screening, Varies for treatment, and at scheduled day 1, day 2 and week 12 visits for population 1 and 2. at scheduled day 1 and week 4, 8, 12, 24, 36, 48, 60, 72, 84, and 96 visits for population 3. for reporting.

Treatment Details

Study Objectives

Study objectives can provide a clearer picture of what you can expect from a treatment.

Primary study objectives

Incidence of treatment-emergent adverse events (TEAEs), serious adverse events (SAEs), AEs leading to treatment discontinuation, and adverse events of interest (AEOIs)

Urine protein/creatinine ratio (UP/C) at week 108

Secondary study objectives

Estimated glomerular filtration rate (eGFR) over 108 weeks

Observed plasma Pharmacokinetic (PK) concentrations

Proportion of subjects achieving complete remission of proteinuria

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Trial Design

3Treatment groups

Experimental Treatment

Group I: Population 3: IgANExperimental Treatment1 Intervention

Subjects with kidney biopsy-confirmed IgAN

Group II: Population 2: IgAN, IgAV, or ASExperimental Treatment1 Intervention

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

Group III: Population 1: FSGS and/or MCDExperimental Treatment1 Intervention

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

0 / 17Eligibility criteria met

Research Highlights

Information in this section is not a recommendation. We encourage patients to speak with their healthcare team when evaluating any treatment decision.

Mechanism Of Action

Side Effect Profile

Prior Approvals

Other Research

Henoch-Schonlein Purpura (HSP) treatments often aim to manage symptoms and prevent renal complications. Sparsentan, a dual endothelin receptor and angiotensin II receptor antagonist, works by blocking endothelin-1 and angiotensin II, which are involved in vasoconstriction, inflammation, and fibrosis. This dual inhibition can reduce proteinuria and protect kidney function, which is vital for HSP patients at risk of chronic kidney disease.

Differential reactivity of cortical and juxtamedullary glomeruli to adenosine-1 and adenosine-2 receptor stimulation and angiotensin-converting enzyme inhibition.Angiotensin-converting enzyme inhibitor treatment early after myocardial infarction attenuates acute cardiac and neuroinflammation without effect on chronic neuroinflammation.Post-stroke losartan and captopril treatments arrest hemorrhagic expansion in SHRsp without lowering blood pressure.