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
Steroid dependence is defined as relapse during tapering of steroid therapy or within 4 weeks of steroid discontinuation.[2]
According to the National Kidney Foundation (NKF) Kidney Disease – Improve Global Outcomes (KGIDO) guidelines in 2012[1], 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]
Cyclophosphamide
- Dose: 2-2.5 mg/kg/d
- Duration: 8 weeks
Cyclophosphamide is contraindicated if fertility is a concern.
Cyclosporine
- Dose: 3-5 mg/kg/d in divided doses
- Duration: 1-2 years
Tacrolimus
- Dose: 0.05-0.1 mg/kg/d in divided doses
- Duration: 1-2 years
Mycophenolate Mofetil (MMF)
- Dose: 500-1000 mg twice daily
- Duration: 1-2 years
Contraindicated medications
Lipoid nephrosis is considered an absolute contraindication to the use of the following medications:
Steroid-Resistance
Steroid resistance is defined as the failure to reach remission despite the use of the above treatment options.[2] In such cases, FSGS must be highly considered and repeat renal biopsy is indicated.[1]
Acute Renal Failure
Patients with MCD complicated with acute renal failure are recommended to reinitiate corticosteroids (similar to regimen of initial therapy) and treated using the appropriate renal replacement therapy.[1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 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.
- ↑ 2.0 2.1 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.