Minimal change disease medical therapy: Difference between revisions
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Pharmacologic therapy using [[corticosteroid]]s 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,<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref> initial empirical treatment using [[corticosteroid]]s in patients presenting with nephrotic syndrome prior to a kidney biopsy is recommended. Notably also, the use of [[statin]]s for [[hyperlipidemia]] and [[ACE-I]] or [[ARB]] for [[proteinuria]] are both not recommended in patients presenting with the initial episode of MCD. | Pharmacologic therapy using [[corticosteroid]]s 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,<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref> initial empirical treatment using [[corticosteroid]]s in patients presenting with nephrotic syndrome prior to a kidney biopsy is recommended. Notably also, the use of [[statin]]s 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 | ==Initial Therapy or Therapy for Infrequent Relapses== | ||
===Prednisone or Prednisolone=== | ===Prednisone or Prednisolone=== | ||
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Relative contraindications of corticosteroids include uncontrolled [[diabetes mellitus]], [[psychiatric disease]]s, and severe [[osteoporosis]]. In such cases, the use of alternative therapy is recommended. | Relative contraindications of corticosteroids include uncontrolled [[diabetes mellitus]], [[psychiatric disease]]s, and severe [[osteoporosis]]. In such cases, the use of alternative therapy is recommended. | ||
==Medical Therapy | ==Medical Therapy== | ||
* According to Children's Nephrotic Syndrome Consensus Conference Pharmacologic medical therapy is recommended among patients with minimal change disease are folowing | * According to Children's Nephrotic Syndrome Consensus Conference Pharmacologic medical therapy is recommended among patients with minimal change disease are folowing | ||
=== Initial therapy === | === Initial therapy === | ||
* '''Pediatric''' | * '''Pediatric''' | ||
** Preferred regimen (1): Prednisone 2 mg/kg per day for six weeks | ** Preferred regimen (1): Prednisone 2 mg/kg per day for six weeks<ref name="pmid23871408" /> | ||
*** Followed by alternate-day prednisone of 1.5 mg/kg for an additional six weeks. | *** Followed by alternate-day prednisone of 1.5 mg/kg for an additional six weeks. | ||
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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
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
- 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[1]
- 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[1]
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 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.