Minimal change disease medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Yazan Daaboul, Serge Korjian

Overview

Pharmacologic therapy using corticosteroids is considered the mainstay of therapy for minimal change disease. According to the National Kidney Foundation (NKF) Kidney Disease – Improve Global Outcomes (KGIDO) guidelines in 2012,[1] initial empirical treatment using corticosteroids in patients presenting with nephrotic syndrome prior to a kidney biopsy is recommended. Notably also, the use of statins for hyperlipidemia and ACE-I or ARB for proteinuria are both not recommended in patients presenting with the initial episode of MCD.

Medical Therapy

  • According to Children's Nephrotic Syndrome Consensus Conference Pharmacologic medical therapy is recommended among patients with minimal change disease are folowing

Initial therapy for children

  •  Pediatric
    • Preferred regimen (1): Prednisone  2 mg/kg per day for six weeks[1]
      • Followed by alternate-day prednisone of 1.5 mg/kg for an additional six weeks.

First relapse

  • Preferred regimen (1): Prednisone  2 mg/kg per day, until the urine protein tests shows negative.

Frequent relapses

  • Preferred regimen (1):  Prednisone therapy of 2 mg/kg, until the urine protein tests shows negative.
    • Followed by alternate-day prednisone of 1.5 mg/kg for four weeks, then tapper to 0.5 mg over a two month period.

Steroid-dependent disease

  • Steroid dependence is defined as relapse during tapering of steroid therapy or within 4 weeks of steroid discontinuation.[2]
  • In the absence of steroid toxicity Prednisone still stands the preferred therapy.
  • In the presence of steroid toxicity in patients with minimal change disease the following drugs may be used to treat the patients.
  • Relative contraindications of corticosteroids include uncontrolled diabetes mellitus, psychiatric diseases, and severe osteoporosis. In such cases, the use of alternative therapy is recommended.
    • Preferred regimen (1): levamisole
    • Preferred regimen (2): Mycophenolate Mofetil (MMF) 500-1000 mg twice daily, for 1-2 years
    • Preferred regimen (2): cyclophosphamide 2-2.5 mg/kg/d for 8 weeks
    • Preferred regimen (3): calcineurin inhibitors (ie, cyclosporine 3-5 mg/kg/d or tacrolimus
  • According to the National Kidney Foundation (NKF) Kidney Disease – Improve Global Outcomes (KGIDO) guidelines in 2012, cyclophosphamide is recommended. In case relapse occurs despite cyclophosphamide or fertility is a concern, cyclosporine or tacrolimus. Mycophenolate mofetil (MMF) may be used, but is often reserved as last option.[1]

Initial therapy for adults

Non Immunosuppressive therapies

  • Adults who are positive with minimal change disease present with edema and most commonly with hypertension.[3]

References

  1. 1.0 1.1 1.2 Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH; et al. (2013). "KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis". Am J Kidney Dis. 62 (3): 403–41. doi:10.1053/j.ajkd.2013.06.002. PMID 23871408.
  2. Waldman M, Crew RJ, Valeri A, Busch J, Stokes B, Markowitz G; et al. (2007). "Adult minimal-change disease: clinical characteristics, treatment, and outcomes". Clin J Am Soc Nephrol. 2 (3): 445–53. doi:10.2215/CJN.03531006. PMID 17699450.
  3. Nakayama M, Katafuchi R, Yanase T, Ikeda K, Tanaka H, Fujimi S (March 2002). "Steroid responsiveness and frequency of relapse in adult-onset minimal change nephrotic syndrome". Am. J. Kidney Dis. 39 (3): 503–12. doi:10.1053/ajkd.2002.31400. PMID 11877569.

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