Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance

Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance (sometimes called SSNS with secondary resistance, or relapsing nephrotic syndrome with acquired steroid resistance) is a kidney disorder that begins in childhood. In this condition, the tiny filtering units in the kidneys — called glomeruli — become leaky and allow 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 around the ankles, belly, and eyes. What makes this condition unique is its two-phase nature. At first, the disease responds well to steroid medicines (like prednisone), which is why it is called 'steroid-sensitive.' However, over time — often after multiple relapses — the kidneys stop responding to steroids as well as they once did. This shift to 'secondary steroid resistance' is a serious development that requires a change in treatment strategy. Key symptoms include puffiness around the eyes (especially in the morning), swelling in the legs and belly, foamy urine, fatigue, and reduced urine output. Treatment typically involves steroids first, then other immune-suppressing medicines such as calcineurin inhibitors (like tacrolimus or cyclosporine), mycophenolate mofetil, or rituximab when resistance develops. Managing this condition requires close monitoring by a kidney specialist (nephrologist) and careful attention to infections, medication side effects, and kidney function over time.

Bring these to your next appointment

• Q1.Why has my child stopped responding to steroids, and what does this mean for their kidneys long-term?,Should we do a kidney biopsy or genetic testing to better understand what is causing the resistance?,What is the next treatment option, and what are the risks and benefits compared to continuing steroids?,How will we know if the new treatment is working, and how long before we expect to see results?,What signs of relapse or complications should prompt me to call you or go to the emergency room?,Are there any clinical trials or newer treatments that my child might be eligible for?,How might this condition affect my child's growth, development, and kidney health as they get older?

Common questions about Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance