Overview
Idiopathic steroid-resistant nephrotic syndrome (SRNS) is a serious kidney disease where the tiny filtering units in the kidneys, called glomeruli, stop working properly. In nephrotic syndrome, the kidneys leak large amounts of protein into the urine instead of keeping it in the blood. Normally, doctors treat nephrotic syndrome with steroid medications like prednisone, and most patients respond well. However, in steroid-resistant nephrotic syndrome, the kidneys do not respond to steroids, making the disease much harder to treat and control. The main symptoms include severe swelling (called edema) in the legs, ankles, face, and belly, caused by low protein levels in the blood. People with this condition also have very foamy or frothy urine due to the protein leaking out. Over time, the kidneys can become permanently damaged, and many patients eventually develop chronic kidney disease or kidney failure. The word 'idiopathic' means the exact cause is not always clear, but in many cases — especially in children — a genetic mutation affecting the kidney filter is responsible. There are many genes that can be involved. Treatment focuses on protecting the kidneys, reducing protein loss, and slowing progression to kidney failure. Kidney transplant may be needed in advanced cases. This condition is also sometimes called steroid-resistant nephrotic syndrome (SRNS) or treatment-resistant nephrotic syndrome.
Also known as:
Key symptoms:
Severe swelling in the legs, ankles, feet, and around the eyesSwollen or puffy bellyFoamy or frothy urineFatigue and low energyReduced amount of urineWeight gain from fluid buildupLoss of appetiteHigh blood pressureIncreased risk of infectionsBlood clots in veins or arteriesHigh cholesterol levelsPale or dull-looking skinDifficulty breathing if fluid builds up around the lungs
Clinical phenotype terms (24)— hover any for plain English
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
Treatments
3 availableSogroya
Idiopathic Short Stature (ISS)
Rubraca
indicated for the treatment of adult patients with a deleterious BRCA mutation (germline and/or somatic)-associated metastatic castration-resistant prostate cancer (mCRPC) who have been treated with a…
indicated for the treatment of adult patients with a deleterious BRCA mutation (germline and/or somatic)-associated metastatic castration-resistant prostate cancer (mCRPC) who have been treated with androgen receptor-directed therapy
Lynparza
for the treatment of adult patients with deleterious or suspected deleterious germline or somatic homologous recombination repair (HRR) gene-mutated metastatic castration-resistant prostate cancer (mC…
for the treatment of adult patients with deleterious or suspected deleterious germline or somatic homologous recombination repair (HRR) gene-mutated metastatic castration-resistant prostate cancer (mCRPC) who have progressed following prior treatment with enzalutamide or abiraterone
Clinical Trials
View all trials with filters →No actively recruiting trials found for Idiopathic steroid-resistant nephrotic syndrome at this time.
New trials open frequently. Follow this disease to get notified.
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
3 resourcesTravel Grants
No travel grants are currently matched to Idiopathic steroid-resistant nephrotic syndrome.
Community
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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 my child or I have genetic testing, and which genes should be tested?,What does the kidney biopsy result mean for my treatment options and long-term outlook?,Which medications will you try first, and how will we know if they are working?,What are the signs that my kidneys are getting worse, and when should I call you or go to the emergency room?,If my kidneys fail, am I a candidate for a kidney transplant, and could the disease come back in the new kidney?,Are there any clinical trials I should know about for SRNS?,What dietary changes do I need to make, and should I see a kidney dietitian?
Common questions about Idiopathic steroid-resistant nephrotic syndrome
What is Idiopathic steroid-resistant nephrotic syndrome?
Idiopathic steroid-resistant nephrotic syndrome (SRNS) is a serious kidney disease where the tiny filtering units in the kidneys, called glomeruli, stop working properly. In nephrotic syndrome, the kidneys leak large amounts of protein into the urine instead of keeping it in the blood. Normally, doctors treat nephrotic syndrome with steroid medications like prednisone, and most patients respond well. However, in steroid-resistant nephrotic syndrome, the kidneys do not respond to steroids, making the disease much harder to treat and control. The main symptoms include severe swelling (called ed
Which specialists treat Idiopathic steroid-resistant nephrotic syndrome?
1 specialists and care centers treating Idiopathic steroid-resistant nephrotic syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Idiopathic steroid-resistant nephrotic syndrome?
1 patient support program are currently tracked on UniteRare for Idiopathic steroid-resistant nephrotic syndrome. See the treatments and support programs sections for copay assistance, eligibility, and contact details.