Steroid-resistant nephrotic syndrome

Steroid-resistant nephrotic syndrome

Steroid-resistant nephrotic syndrome (SRNS) is a condition characterized by kidney damage that leads to heavy proteinuria (excessive protein in the urine), hypoalbuminemia (low levels of albumin in the blood), edema (swelling), and often high cholesterol levels. It’s called “steroid-resistant” because the condition does not respond to treatment with corticosteroid medications, which are typically the first-line treatment for nephrotic syndrome.

Here are some key points about SRNS:

  1. Causes: SRNS can have various underlying causes, including genetic mutations, autoimmune diseases, infections, certain medications, and other kidney diseases. In many cases, the exact cause remains unknown.
  2. Diagnosis: Diagnosis of SRNS involves clinical evaluation, laboratory tests (such as urine tests for proteinuria and blood tests for serum albumin levels), imaging studies (such as ultrasound or CT scan of the kidneys), and sometimes a kidney biopsy to determine the underlying cause and extent of kidney damage.
  3. Treatment: Managing SRNS can be challenging because it doesn’t respond to standard corticosteroid therapy. Treatment strategies for SRNS may include:
  • Immunosuppressive therapy: Other medications that suppress the immune system may be prescribed, such as calcineurin inhibitors (e.g., cyclosporine, tacrolimus), mycophenolate mofetil, rituximab, or other agents.
  • Supportive therapy: This may include medications to control blood pressure, reduce edema (swelling), and manage complications such as high cholesterol levels and blood clots.
  • Dietary and lifestyle modifications: These may include dietary changes to manage proteinuria and reduce salt intake, as well as regular exercise and weight management.
  1. Prognosis: The prognosis for SRNS varies depending on factors such as the underlying cause, the severity of kidney damage, and the response to treatment. Some cases of SRNS may progress to end-stage kidney disease (ESKD) requiring dialysis or kidney transplantation, while others may remain stable with appropriate treatment.
  2. Genetic Testing: In some cases, SRNS may be caused by genetic mutations that affect kidney function. Genetic testing may be recommended for individuals with SRNS, especially in cases where there is a family history of kidney disease or if the condition presents at a young age.

Overall, SRNS requires careful management by a multidisciplinary team of healthcare providers, including nephrologists, pediatricians (if the condition occurs in children), and possibly genetic counselors. Treatment strategies are aimed at slowing disease progression, managing symptoms, and preserving kidney function for as long as possible.