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Immunosuppressive therapy in children with primary nephrotic syndrome: single center experience, Karachi, Pakistan

Khemchand Netaram Moorani1,2, Harnam Moolchand Hotchandani3, Aasia Mohammad Zubair4,3

  • 1Department of Pediatric Nephrology, National Institute of Child Health (NICH), Jinnah Sindh Medical University, Karachi, Pakistan. khemchandn@hotmail.com.

BMC Nephrology
|July 5, 2019
PubMed

Insights

Treatment for difficult nephrotic syndrome in children involves various immunosuppressive agents. Levamisole and cyclophosphamide effectively maintain remission in frequent relapsing/steroid-dependent cases, while cyclosporine aids remission in steroid-resistant nephrotic syndrome.

Area of Science:

  • Pediatric Nephrology
  • Immunosuppressive Therapy
  • Nephrotic Syndrome

Background:

  • Nephrotic syndrome in children is often steroid-sensitive, but difficult-to-treat cases (frequent relapsing, steroid-dependent, steroid-resistant) pose therapeutic challenges.
  • Common treatment options include low-dose steroids, levamisole, cyclophosphamide (CPM), mycophenolate mofetil (MMF), and calcineurin inhibitors (CNIs).

Purpose of the Study:

  • To evaluate the efficacy of steroid therapy and alternative immunosuppressive agents (ISAs) in children diagnosed with difficult nephrotic syndrome (DNS).

Main Methods:

  • A retrospective cohort study included 176 children with DNS managed over 12 years (2005-2017).
  • Treatment protocols involved initial oral prednisolone (OP), followed by low-dose OP, levamisole, or cyclophosphamide for frequent relapsing/steroid-dependent cases.
  • Steroid-resistant cases and non-responders to levamisole/cyclophosphamide underwent biopsy and were treated with CNIs and MMF.

Main Results:

  • The study identified 130 (73.86%) children with frequent relapsing/steroid-dependent (FR/SD) and 46 (26.13%) with steroid-resistant nephrotic syndrome (SRNS).
  • Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) were the primary histological findings.
  • Levamisole showed 80% effectiveness, CPM achieved 57.77% complete remission (CR) and 22.22% partial remission (PR), cyclosporine (CS) induced 46.59% CR and 39.77% PR, and MMF resulted in 69% PR and 12.82% CR.
  • At follow-up, 46% maintained remission off-treatment, 35% on therapy, 5.68% progressed to chronic kidney disease, and mortality was 2.84%.

Conclusions:

  • Levamisole and cyclophosphamide are effective in maintaining remission for FR/SD nephrotic syndrome.
  • Cyclosporine demonstrates efficacy in inducing remission for SRNS.
  • FSGS is a significant factor contributing to resistance against both steroid and alternative ISAs.
Abstract

Keywords:
CyclophosphamideCyclosporinLevamisoleMinimal change diseaseMycophenolate mofitilNephrotic syndromeOral prednisolone

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