Overview
IgG4-related kidney disease (also called IgG4-related renal disease or IgG4-RKD) is a rare condition where the immune system mistakenly attacks the kidneys. It is part of a broader group of conditions called IgG4-related disease (IgG4-RD), in which a specific type of antibody called immunoglobulin G4 (IgG4) builds up in various organs and causes inflammation and scarring. When the kidneys are affected, the most common form is called tubulointerstitial nephritis, where the tissue between the kidney's filtering tubes becomes inflamed and swollen. This can lead to reduced kidney function over time. Patients may notice symptoms such as decreased urine output, swelling in the legs or around the eyes, fatigue, and sometimes flank pain. Many people have no obvious symptoms early on, and the disease is often discovered through abnormal blood tests or imaging done for other reasons. Some patients also have involvement of other organs, such as the pancreas, salivary glands, or lymph nodes. The good news is that IgG4-related kidney disease usually responds well to treatment with corticosteroids (such as prednisone), which reduce inflammation and can restore kidney function if caught early. For patients who relapse or cannot tolerate steroids, other immune-suppressing medications like rituximab, azathioprine, or mycophenolate mofetil may be used. Early diagnosis and treatment are important to prevent permanent kidney damage.
Key symptoms:
Decreased kidney functionSwelling in the legs, ankles, or around the eyesFatigue and general tirednessReduced urine outputFlank or back painUnexplained weight lossElevated blood creatinine levels found on routine blood testsProtein in the urineKidney masses or swelling seen on imagingSwollen salivary glands or dry mouthSwollen lymph nodesSymptoms from other organ involvement such as abdominal pain from pancreas inflammation
Clinical phenotype terms (50)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for IgG4-related kidney disease.
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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
Travel Grants
No travel grants are currently matched to IgG4-related kidney disease.
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Caregiver Resources
NORD Caregiver 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 has my kidney function been affected, and is the damage reversible?,Are any of my other organs affected by IgG4-related disease?,What is the plan for tapering steroids, and what are the risks of relapse?,Should I be treated with rituximab or another steroid-sparing medication?,How often will I need blood tests and follow-up imaging?,Are there any dietary changes I should make to protect my kidneys?,What symptoms should prompt me to seek urgent medical care?
Common questions about IgG4-related kidney disease
What is IgG4-related kidney disease?
IgG4-related kidney disease (also called IgG4-related renal disease or IgG4-RKD) is a rare condition where the immune system mistakenly attacks the kidneys. It is part of a broader group of conditions called IgG4-related disease (IgG4-RD), in which a specific type of antibody called immunoglobulin G4 (IgG4) builds up in various organs and causes inflammation and scarring. When the kidneys are affected, the most common form is called tubulointerstitial nephritis, where the tissue between the kidney's filtering tubes becomes inflamed and swollen. This can lead to reduced kidney function over tim
How is IgG4-related kidney disease inherited?
IgG4-related kidney disease follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does IgG4-related kidney disease typically begin?
Typical onset of IgG4-related kidney disease is adult. Age of onset can vary across affected individuals.
Which specialists treat IgG4-related kidney disease?
18 specialists and care centers treating IgG4-related kidney disease are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.