Overview
Idiopathic steroid-resistant nephrotic syndrome with sensitivity to second-line immunosuppressive therapy (sometimes called SRNS with second-line response) is a rare kidney disease. In nephrotic syndrome, the tiny filtering units in the kidneys — called glomeruli — become damaged and leaky. This causes large amounts of protein to spill into the urine instead of staying in the blood where it belongs. When this happens, the body loses important proteins, leading to swelling (especially in the legs, ankles, and around the eyes), low protein levels in the blood, and high cholesterol. What makes this specific form unique is a two-part pattern: first, the disease does not respond to steroids (the usual first treatment for nephrotic syndrome). This is called steroid resistance. However, unlike some other steroid-resistant forms, this condition does respond to second-line immunosuppressive medicines — drugs that calm down an overactive immune system, such as calcineurin inhibitors like cyclosporine or tacrolimus. Symptoms include significant swelling, foamy urine (from protein), fatigue, reduced urine output, and weight gain from fluid buildup. Because the cause is not fully understood (hence 'idiopathic'), management focuses on finding the right combination of immune-suppressing medicines. With the right treatment, many patients can achieve remission and protect their kidneys from long-term damage.
Also known as:
Key symptoms:
Severe swelling in the legs, ankles, feet, and around the eyesFoamy or frothy urine caused by excess proteinUnexplained weight gain from fluid buildup in the bodyLow levels of protein in the bloodHigh cholesterol and high triglyceridesFatigue and low energyReduced amount of urinePuffy abdomen from fluid accumulationIncreased risk of infections due to loss of immune proteinsIncreased risk of blood clots
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Idiopathic steroid-resistant nephrotic syndrome with sensitivity to second-line immunosuppressive therapy.
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Specialists
View all specialists →No specialists are currently listed for Idiopathic steroid-resistant nephrotic syndrome with sensitivity to second-line immunosuppressive therapy.
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 steroid-resistant nephrotic syndrome with sensitivity to second-line immunosuppressive therapy.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
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.Which second-line immunosuppressive medicine do you recommend for me, and why?,Should I have genetic testing to find out if there is a genetic cause for my nephrotic syndrome?,How will we know if the treatment is working, and how long before we expect to see improvement?,What are the long-term side effects of the medicines I will be taking, and how will we monitor for them?,What are the signs of a relapse, and what should I do if I think I am having one?,Are there any clinical trials or newer treatments I should know about?,What dietary changes should I make, and should I see a kidney dietitian?
Common questions about Idiopathic steroid-resistant nephrotic syndrome with sensitivity to second-line immunosuppressive therapy
What is Idiopathic steroid-resistant nephrotic syndrome with sensitivity to second-line immunosuppressive therapy?
Idiopathic steroid-resistant nephrotic syndrome with sensitivity to second-line immunosuppressive therapy (sometimes called SRNS with second-line response) is a rare kidney disease. In nephrotic syndrome, the tiny filtering units in the kidneys — called glomeruli — become damaged and leaky. This causes large amounts of protein to spill into the urine instead of staying in the blood where it belongs. When this happens, the body loses important proteins, leading to swelling (especially in the legs, ankles, and around the eyes), low protein levels in the blood, and high cholesterol. What makes t