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.
Initial Therapy or Therapy for Infrequent Relapses[1]
Prednisone or Prednisolone
Prednisone and prednisolone are considered equivalent and may be used in same dosage.
- Dose: Daily single dose of 1 mg/kg/d up to 80 mg /day or alternate-day single dose of 2 mg/kg/d up to 120 mg/day
- Duration: 4 weeks, if tolerated. Treatment may continue up to 16 weeks to achieve remission, only if tolerated. Treatment should be tapered slowly over 6 months after achieving remission.
Relative contraindications of corticosteroids include uncontrolled diabetes mellitus, psychiatric diseases, and severe osteoporosis. In such cases, the use of alternative therapy is recommended.
Medical Therapy[1]
- According to Children's Nephrotic Syndrome Consensus Conference Pharmacologic medical therapy is recommended among patients with minimal change disease are folowing
Initial therapy
- Pediatric
- Preferred regimen (1): Prednisone 2 mg/kg per day for six weeks
- Followed by alternate-day prednisone of 1.5 mg/kg for an additional six weeks.
- Preferred regimen (1): Prednisone 2 mg/kg per day for 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
- 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
- Cyclophosphamide is contraindicated if fertility is of a concern
- 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]
References
- ↑ 1.0 1.1 1.2 1.3 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.
- ↑ 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.