Minimal change disease medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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.
Frequent Relapses or Steroid-Dependence[1]
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. Mycofenolate 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.