Overview
Immunoglobulin A nephropathy, also called IgA nephropathy or Berger's disease, is a kidney disease where a protein called IgA — normally part of your immune system — builds up inside the tiny filtering units of the kidneys (called glomeruli). This buildup causes inflammation that slowly damages the kidneys over time. IgA is an antibody your body makes to fight infections, but in this disease, abnormal forms of IgA get trapped in the kidneys and trigger an immune response that harms kidney tissue. Many people with IgA nephropathy notice blood in their urine, especially during or after a cold or throat infection. Some people also have foamy urine from protein leaking out, swelling in the legs or ankles, and high blood pressure. Symptoms can be very mild at first, and some people are only diagnosed after a routine urine test shows abnormalities. Treatment focuses on slowing kidney damage and controlling blood pressure. Medications like ACE inhibitors or ARBs are commonly used to protect the kidneys. In 2023, the FDA approved sparsentan (Filspari) specifically for IgA nephropathy — the first targeted therapy for this disease. Some patients also receive corticosteroids or other immune-suppressing drugs. A small number of patients eventually develop kidney failure and may need dialysis or a kidney transplant. Early diagnosis and treatment give the best chance of preserving kidney function long-term.
Also known as:
Key symptoms:
Blood in the urine (urine looks pink, red, or cola-colored)Foamy or bubbly urine from protein leaking into the urineSwelling in the hands, feet, ankles, or legsHigh blood pressureFlank or back pain around the kidney areaFatigue and low energyFrequent episodes of blood in the urine during or after a cold or throat infectionDecreased urine output in advanced casesHeadaches related to high blood pressureNausea or loss of appetite in later stages when kidney function declines
Clinical phenotype terms (17)— hover any for plain English
Multifactorial
Caused by a mix of several genes and environmental factors
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
10 eventsNovartis Pharmaceuticals — PHASE3
Hinge Bio — PHASE1
Regeneron Pharmaceuticals — PHASE1
Radboud University Medical Center — PHASE2, PHASE3
Shanghai Henlius Biotech — PHASE2
Alexion Pharmaceuticals, Inc. — PHASE3
Novartis Pharmaceuticals — PHASE2
Christian Medical College, Vellore, India — PHASE4
Filspari: FDA approved
to slow kidney function decline in adults with primary immunoglobulin A nephropathy (IgAN) who are at risk for disease progression
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
5 availableVanrafia
indicated to reduce proteinuria in adults with primary immunoglobulin A nephropathy (IgAN) at risk of rapid disease progression, generally a urine protein-to-creatinine ratio (UPCR) ≥ 1.5 g/g
Tarpeyo
TARPEYO is indicated to reduce the loss of kidney function in adults with primary immunoglobulin A nephropathy (IgAN) who are at risk for disease progression.
Voyxact
indicated to reduce proteinuria in adults with primary immunoglobulin A nephropathy (IgAN) at risk for disease progression
Filspari
to slow kidney function decline in adults with primary immunoglobulin A nephropathy (IgAN) who are at risk for disease progression
FABHALTA
the reduction of proteinuria in adults with primary immunoglobulin A nephropathy (IgAN) at risk of rapid disease progression, generally a urine protein-to-creatinine ratio (UPCR) ≥ 1.5 g/g
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
5 resourcesTravel Grants
No travel grants are currently matched to Immunoglobulin A nephropathy.
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1 articlesCaregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How much kidney function have I already lost, and what is my current eGFR?,Should I start sparsentan or an ACE inhibitor/ARB, and what are the pros and cons for my situation?,How often do I need blood and urine tests, and what numbers should I watch most closely?,What diet changes will help protect my kidneys — how much protein and salt should I eat?,What is my risk of eventually needing dialysis or a kidney transplant based on my biopsy results?,Are there any clinical trials I should consider for newer IgA nephropathy treatments?,Should other family members be tested, and is there a genetic component in my case?
Common questions about Immunoglobulin A nephropathy
What is Immunoglobulin A nephropathy?
Immunoglobulin A nephropathy, also called IgA nephropathy or Berger's disease, is a kidney disease where a protein called IgA — normally part of your immune system — builds up inside the tiny filtering units of the kidneys (called glomeruli). This buildup causes inflammation that slowly damages the kidneys over time. IgA is an antibody your body makes to fight infections, but in this disease, abnormal forms of IgA get trapped in the kidneys and trigger an immune response that harms kidney tissue. Many people with IgA nephropathy notice blood in their urine, especially during or after a cold o
How is Immunoglobulin A nephropathy inherited?
Immunoglobulin A nephropathy follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Immunoglobulin A nephropathy?
Yes — 9 recruiting clinical trials are currently listed for Immunoglobulin A nephropathy on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Immunoglobulin A nephropathy?
25 specialists and care centers treating Immunoglobulin A nephropathy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Immunoglobulin A nephropathy?
2 patient support programs are currently tracked on UniteRare for Immunoglobulin A nephropathy. See the treatments and support programs sections for copay assistance, eligibility, and contact details.