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* In MCD patients who are treated with low-dose [[prednisone]] had shown [[Remission (medicine)|remission]] of [[proteinuria]] in 75% of the patients.<ref name="pmid49167902">{{cite journal |vauthors=Black DA, Rose G, Brewer DB |title=Controlled trial of prednisone in adult patients with the nephrotic syndrome |journal=Br Med J |volume=3 |issue=5720 |pages=421–6 |date=August 1970 |pmid=4916790 |pmc=1701394 |doi= |url=}}</ref> | * In MCD patients who are treated with low-dose [[prednisone]] had shown [[Remission (medicine)|remission]] of [[proteinuria]] in 75% of the patients.<ref name="pmid49167902">{{cite journal |vauthors=Black DA, Rose G, Brewer DB |title=Controlled trial of prednisone in adult patients with the nephrotic syndrome |journal=Br Med J |volume=3 |issue=5720 |pages=421–6 |date=August 1970 |pmid=4916790 |pmc=1701394 |doi= |url=}}</ref> | ||
'''Rituximab''' | '''Rituximab''' | ||
* Rituximab is used in both adults and children patients who are positive for minimal change disease.<ref name="pmid23338210">{{cite journal |vauthors=Sinha A, Bagga A |title=Rituximab therapy in nephrotic syndrome: implications for patients' management |journal=Nat Rev Nephrol |volume=9 |issue=3 |pages=154–69 |date=March 2013 |pmid=23338210 |doi=10.1038/nrneph.2012.289 |url=}}</ref> | * Rituximab is used in both adults and children patients who are positive for minimal change disease.<ref name="pmid23338210">{{cite journal |vauthors=Sinha A, Bagga A |title=Rituximab therapy in nephrotic syndrome: implications for patients' management |journal=Nat Rev Nephrol |volume=9 |issue=3 |pages=154–69 |date=March 2013 |pmid=23338210 |doi=10.1038/nrneph.2012.289 |url=}}</ref><ref name="pmid21762648">{{cite journal |vauthors=Hoxha E, Stahl RA, Harendza S |title=Rituximab in adult patients with immunosuppressive-dependent minimal change disease |journal=Clin. Nephrol. |volume=76 |issue=2 |pages=151–8 |date=August 2011 |pmid=21762648 |doi= |url=}}</ref> | ||
* A human monoclonal antibody that identifies the CD20 on B-lymphocytes.<ref name="pmid23739238">{{cite journal |vauthors=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 |title=Rituximab is a safe and effective long-term treatment for children with steroid and calcineurin inhibitor-dependent idiopathic nephrotic syndrome |journal=Kidney Int. |volume=84 |issue=5 |pages=1025–33 |date=November 2013 |pmid=23739238 |pmc=3816123 |doi=10.1038/ki.2013.211 |url=}}</ref><ref name="pmid18465150">{{cite journal |vauthors=Guigonis V, Dallocchio A, Baudouin V, Dehennault M, Hachon-Le Camus C, Afanetti M, Groothoff J, Llanas B, Niaudet P, Nivet H, Raynaud N, Taque S, Ronco P, Bouissou F |title=Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases |journal=Pediatr. Nephrol. |volume=23 |issue=8 |pages=1269–79 |date=August 2008 |pmid=18465150 |doi=10.1007/s00467-008-0814-1 |url=}}</ref><ref name="pmid21566104">{{cite journal |vauthors=Ravani P, Magnasco A, Edefonti A, Murer L, Rossi R, Ghio L, Benetti E, Scozzola F, Pasini A, Dallera N, Sica F, Belingheri M, Scolari F, Ghiggeri GM |title=Short-term effects of rituximab in children with steroid- and calcineurin-dependent nephrotic syndrome: a randomized controlled trial |journal=Clin J Am Soc Nephrol |volume=6 |issue=6 |pages=1308–15 |date=June 2011 |pmid=21566104 |pmc=3109926 |doi=10.2215/CJN.09421010 |url=}}</ref> | * A human monoclonal antibody that identifies the CD20 on B-lymphocytes.<ref name="pmid23739238">{{cite journal |vauthors=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 |title=Rituximab is a safe and effective long-term treatment for children with steroid and calcineurin inhibitor-dependent idiopathic nephrotic syndrome |journal=Kidney Int. |volume=84 |issue=5 |pages=1025–33 |date=November 2013 |pmid=23739238 |pmc=3816123 |doi=10.1038/ki.2013.211 |url=}}</ref><ref name="pmid18465150">{{cite journal |vauthors=Guigonis V, Dallocchio A, Baudouin V, Dehennault M, Hachon-Le Camus C, Afanetti M, Groothoff J, Llanas B, Niaudet P, Nivet H, Raynaud N, Taque S, Ronco P, Bouissou F |title=Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases |journal=Pediatr. Nephrol. |volume=23 |issue=8 |pages=1269–79 |date=August 2008 |pmid=18465150 |doi=10.1007/s00467-008-0814-1 |url=}}</ref><ref name="pmid21566104">{{cite journal |vauthors=Ravani P, Magnasco A, Edefonti A, Murer L, Rossi R, Ghio L, Benetti E, Scozzola F, Pasini A, Dallera N, Sica F, Belingheri M, Scolari F, Ghiggeri GM |title=Short-term effects of rituximab in children with steroid- and calcineurin-dependent nephrotic syndrome: a randomized controlled trial |journal=Clin J Am Soc Nephrol |volume=6 |issue=6 |pages=1308–15 |date=June 2011 |pmid=21566104 |pmc=3109926 |doi=10.2215/CJN.09421010 |url=}}</ref> | ||
Revision as of 19:38, 12 June 2018
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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
- Rituximab is used in both adults and children patients who are positive for minimal change disease.[15][16]
- Rituximab is used in the treatment on another diseases along with MCD, membranous nephropathy, FSGS.
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- In severe steroid-dependent minimal change disease patients rituximab is efficient and safe.[20]
- In MCD patients rituximab may be considered as a radical therapeutic agent for patients with steroid-dependent MCNS.[21]
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.
- ↑ Sinha A, Bagga A (March 2013). "Rituximab therapy in nephrotic syndrome: implications for patients' management". Nat Rev Nephrol. 9 (3): 154–69. doi:10.1038/nrneph.2012.289. PMID 23338210.
- ↑ Hoxha E, Stahl RA, Harendza S (August 2011). "Rituximab in adult patients with immunosuppressive-dependent minimal change disease". Clin. Nephrol. 76 (2): 151–8. PMID 21762648.
- ↑ 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.
- ↑ Guigonis V, Dallocchio A, Baudouin V, Dehennault M, Hachon-Le Camus C, Afanetti M, Groothoff J, Llanas B, Niaudet P, Nivet H, Raynaud N, Taque S, Ronco P, Bouissou F (August 2008). "Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases". Pediatr. Nephrol. 23 (8): 1269–79. doi:10.1007/s00467-008-0814-1. PMID 18465150.
- ↑ Ravani P, Magnasco A, Edefonti A, Murer L, Rossi R, Ghio L, Benetti E, Scozzola F, Pasini A, Dallera N, Sica F, Belingheri M, Scolari F, Ghiggeri GM (June 2011). "Short-term effects of rituximab in children with steroid- and calcineurin-dependent nephrotic syndrome: a randomized controlled trial". Clin J Am Soc Nephrol. 6 (6): 1308–15. doi:10.2215/CJN.09421010. PMC 3109926. PMID 21566104.
- ↑ Munyentwali H, Bouachi K, Audard V, Remy P, Lang P, Mojaat R, Deschênes G, Ronco PM, Plaisier EM, Dahan KY (March 2013). "Rituximab is an efficient and safe treatment in adults with steroid-dependent minimal change disease". Kidney Int. 83 (3): 511–6. doi:10.1038/ki.2012.444. PMID 23325085.
- ↑ Iwabuchi Y, Takei T, Moriyama T, Itabashi M, Nitta K (December 2014). "Long-term prognosis of adult patients with steroid-dependent minimal change nephrotic syndrome following rituximab treatment". Medicine (Baltimore). 93 (29): e300. doi:10.1097/MD.0000000000000300. PMC 4602588. PMID 25546674.