Overview
Idiopathic multidrug-resistant nephrotic syndrome (also called idiopathic steroid-resistant nephrotic syndrome or SRNS) is a serious kidney disease where the tiny filtering units in the kidneys, called glomeruli, stop working properly. In a healthy kidney, these filters keep important proteins — especially one called albumin — inside the blood. In this condition, the filters become leaky, allowing large amounts of protein to spill into the urine. This leads to low protein levels in the blood, which causes fluid to build up in the body, especially in the legs, belly, and around the eyes. What makes this condition particularly challenging is that it does not respond to the standard first-line treatment, steroids (like prednisone), and also fails to respond to several other immunosuppressive medications. This is why it is called 'multidrug-resistant.' The exact cause is often unknown — which is why the word 'idiopathic' is used — though in many cases, genetic changes in genes that control kidney filter function are found. Symptoms include severe swelling, foamy urine, fatigue, and high blood pressure. Over time, the kidneys can lose more and more function, and many patients eventually develop chronic kidney disease or kidney failure, requiring dialysis or a kidney transplant. Management focuses on reducing protein loss, protecting kidney function, controlling blood pressure, and preventing complications. This is a rare and complex condition that requires care from a specialist kidney doctor (nephrologist).
Key symptoms:
Severe swelling (edema), especially in the legs, ankles, feet, belly, and around the eyesFoamy or frothy urine due to high protein levelsWeight gain from fluid buildupFatigue and low energyHigh blood pressureReduced urine outputLoss of appetitePale, puffy appearanceIncreased risk of infectionsBlood clots in veins or arteriesHigh cholesterol and triglyceride levels in the bloodMuscle cramps or weakness from low blood protein
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
9 eventsNational Human Genome Research Institute (NHGRI) — PHASE2
Boehringer Ingelheim — PHASE3
Akebia Therapeutics — PHASE2
Sanofi — PHASE2
Yale University — PHASE1, PHASE2
University of Michigan
Kaneka Medical America LLC — NA
Travere Therapeutics, Inc. — PHASE3
Kaneka Medical America LLC — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Idiopathic multidrug-resistant nephrotic syndrome.
9 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Specialists
View all specialists →No specialists are currently listed for Idiopathic multidrug-resistant nephrotic syndrome.
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 Idiopathic multidrug-resistant nephrotic syndrome.
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
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.Should I have genetic testing, and which genes should be included in the panel?,What type of kidney damage was found on my biopsy, and what does that mean for my outlook?,Which medications are you recommending, and what are the risks and benefits of each?,How quickly might my kidney function decline, and what signs should I watch for at home?,What dietary changes do I need to make, and should I see a renal dietitian?,At what point should we start discussing dialysis or kidney transplantation?,Are there any clinical trials I might be eligible for?
Common questions about Idiopathic multidrug-resistant nephrotic syndrome
What is Idiopathic multidrug-resistant nephrotic syndrome?
Idiopathic multidrug-resistant nephrotic syndrome (also called idiopathic steroid-resistant nephrotic syndrome or SRNS) is a serious kidney disease where the tiny filtering units in the kidneys, called glomeruli, stop working properly. In a healthy kidney, these filters keep important proteins — especially one called albumin — inside the blood. In this condition, the filters become leaky, allowing large amounts of protein to spill into the urine. This leads to low protein levels in the blood, which causes fluid to build up in the body, especially in the legs, belly, and around the eyes. What
Are there clinical trials for Idiopathic multidrug-resistant nephrotic syndrome?
Yes — 9 recruiting clinical trials are currently listed for Idiopathic multidrug-resistant nephrotic syndrome on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.