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: Vamsikrishna Gunnam M.B.B.S [2]
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 following
Initial therapy for children
- Pediatric
- Preferred regimen (1): Prednisone 2 mg/kg per day for six weeks[1][2]
- 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][2]
First relapse
- Preferred regimen (1): Prednisone 2 mg/kg per day, until the urine protein tests shows negative.[3]
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.[4][5][6]
- 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 (3): 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]
Initial therapy for adults
- Adults who are positive with minimal change disease present with edema and most commonly with hypertension.[7][8]
- First-line therapy in adults with minimal change disease low-sodium diet and diuretics for fluid removal.
- Angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blocker (ARB) are of good choice to control hypertension.And by using these drugs have an additional benefit like reducing urinary protein excretion.[9]
Non Immunosuppressive therapies
- Glucocorticoid therapy with prednisone or prednisolone in minimal change disease(MCD) patients.[10][11][12]
- Glucocorticoid have antiproteinuric effect on the glomerular filtration barrier apart from the immunosuppressive effect.[13]
- Preferred regimen (1): prednisone 60 mg/day for eight weeks.
- In MCD patients who are treated with low-dose prednisone had shown remission of proteinuria in 75% of the patients.[14]
Rituximab
- A human monoclonal antibody that identifies the CD20 on B-lymphocytes.[15]
- Rituximab is used in the treatment on another diseases along with MCD, membranous nephropathy, FSGS.
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.
- ↑ Vivarelli M, Massella L, Ruggiero B, Emma F (February 2017). "Minimal Change Disease". Clin J Am Soc Nephrol. 12 (2): 332–345. doi:10.2215/CJN.05000516. PMC 5293332. PMID 27940460.
- ↑ Vivarelli, Marina; Massella, Laura; Ruggiero, Barbara; Emma, Francesco (2017). "Minimal Change Disease". Clinical Journal of the American Society of Nephrology. 12 (2): 332–345. doi:10.2215/CJN.05000516. ISSN 1555-9041.
- ↑ 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.
- ↑ Vivarelli, Marina; Massella, Laura; Ruggiero, Barbara; Emma, Francesco (2017). "Minimal Change Disease". Clinical Journal of the American Society of Nephrology. 12 (2): 332–345. doi:10.2215/CJN.05000516. ISSN 1555-9041.
- ↑ Vivarelli M, Massella L, Ruggiero B, Emma F (February 2017). "Minimal Change Disease". Clin J Am Soc Nephrol. 12 (2): 332–345. doi:10.2215/CJN.05000516. PMC 5293332. PMID 27940460.
- ↑ 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.
- ↑ Mak SK, Short CD, Mallick NP (November 1996). "Long-term outcome of adult-onset minimal-change nephropathy". Nephrol. Dial. Transplant. 11 (11): 2192–201. PMID 8941578.
- ↑ Galle J (July 2008). "Reduction of proteinuria with angiotensin receptor blockers". Nat Clin Pract Cardiovasc Med. 5 Suppl 1: S36–43. doi:10.1038/ncpcardio0806. PMID 18580865.
- ↑ Meyrier AY (September 2009). "Treatment of focal segmental glomerulosclerosis with immunophilin modulation: when did we stop thinking about pathogenesis?". Kidney Int. 76 (5): 487–91. doi:10.1038/ki.2009.204. PMID 19494796.
- ↑ Hogan J, Radhakrishnan J (April 2013). "The treatment of minimal change disease in adults". J. Am. Soc. Nephrol. 24 (5): 702–11. doi:10.1681/ASN.2012070734. PMID 23431071.
- ↑ Nolasco F, Cameron JS, Heywood EF, Hicks J, Ogg C, Williams DG (June 1986). "Adult-onset minimal change nephrotic syndrome: a long-term follow-up". Kidney Int. 29 (6): 1215–23. PMID 3747335.
- ↑ Black DA, Rose G, Brewer DB (August 1970). "Controlled trial of prednisone in adult patients with the nephrotic syndrome". Br Med J. 3 (5720): 421–6. PMC 1701394. PMID 4916790.
- ↑ Black DA, Rose G, Brewer DB (August 1970). "Controlled trial of prednisone in adult patients with the nephrotic syndrome". Br Med J. 3 (5720): 421–6. PMC 1701394. PMID 4916790.
- ↑ Ravani P, Ponticelli A, Siciliano C, Fornoni A, Magnasco A, Sica F, Bodria M, Caridi G, Wei C, Belingheri M, Ghio L, Merscher-Gomez S, Edefonti A, Pasini A, Montini G, Murtas C, Wang X, Muruve D, Vaglio A, Martorana D, Pani A, Scolari F, Reiser J, Ghiggeri GM (November 2013). "Rituximab is a safe and effective long-term treatment for children with steroid and calcineurin inhibitor-dependent idiopathic nephrotic syndrome". Kidney Int. 84 (5): 1025–33. doi:10.1038/ki.2013.211. PMC 3816123. PMID 23739238.