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==Overview== | ==Overview== | ||
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. | ||
==Medical Therapy== | ==Medical Therapy== | ||
* According to Children's Nephrotic Syndrome Consensus Conference Pharmacologic medical therapy is recommended among patients with minimal change disease are | * 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 === | === Initial therapy for children === | ||
* '''Pediatric''' | * '''Pediatric''' | ||
** Preferred regimen (1): | ** 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. | ||
==== First relapse ==== | ==== First relapse ==== | ||
* Preferred regimen (1): | * Preferred regimen (1): [[Prednisone]] 2 mg/kg per day, until the urine protein tests shows negative. | ||
==== Frequent relapses ==== | ==== Frequent relapses ==== | ||
* Preferred regimen (1): | * 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. | ** 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-dependent disease ==== | ||
* Steroid dependence is defined as relapse during tapering of steroid therapy or within 4 weeks of steroid discontinuation.<ref name="pmid17699450">{{cite journal| author=Waldman M, Crew RJ, Valeri A, Busch J, Stokes B, Markowitz G et al.| title=Adult minimal-change disease: clinical characteristics, treatment, and outcomes. | journal=Clin J Am Soc Nephrol | year= 2007 | volume= 2 | issue= 3 | pages= 445-53 | pmid=17699450 | doi=10.2215/CJN.03531006 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17699450 }} </ref> | * [[Steroid]] dependence is defined as relapse during tapering of steroid therapy or within 4 weeks of [[steroid]] discontinuation.<ref name="pmid17699450">{{cite journal| author=Waldman M, Crew RJ, Valeri A, Busch J, Stokes B, Markowitz G et al.| title=Adult minimal-change disease: clinical characteristics, treatment, and outcomes. | journal=Clin J Am Soc Nephrol | year= 2007 | volume= 2 | issue= 3 | pages= 445-53 | pmid=17699450 | doi=10.2215/CJN.03531006 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17699450 }} </ref> | ||
* In the absence of steroid toxicity Prednisone still stands the preferred therapy. | * 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. | * 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 disease]]s, and severe [[osteoporosis]]. In such cases, the use of alternative therapy is recommended. | * Relative [[Contraindication|contraindications]] of [[Corticosteroid|corticosteroids]] include uncontrolled [[diabetes mellitus]], [[psychiatric disease]]s, and severe [[osteoporosis]]. In such cases, the use of alternative therapy is recommended. | ||
** Preferred regimen (1): | ** Preferred regimen (1): [[levamisole]] | ||
** Preferred regimen (2): Mycophenolate Mofetil (MMF) 500-1000 mg twice daily, for 1-2 years | ** Preferred regimen (2): [[Mycophenolate]] Mofetil (MMF) 500-1000 mg twice daily, for 1-2 years | ||
** Preferred regimen (2): | ** Preferred regimen (2): [[cyclophosphamide]] 2-2.5 mg/kg/d for 8 weeks | ||
*** [[Cyclophosphamide]] is contraindicated if [[fertility]] is of a concern | *** [[Cyclophosphamide]] is contraindicated if [[fertility]] is of a concern | ||
** Preferred regimen (3): [[Calcineurin inhibitor|calcineurin]] inhibitors (ie, [[cyclosporine]] 3-5 mg/kg/d or tacrolimus | ** Preferred regimen (3): [[Calcineurin inhibitor|calcineurin]] inhibitors (ie, [[cyclosporine]] 3-5 mg/kg/d or tacrolimus | ||
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=== Initial therapy for adults === | === Initial therapy for adults === | ||
* Adults who are positive with minimal change disease present with edema and most commonly with hypertension.<ref name="pmid11877569">{{cite journal |vauthors=Nakayama M, Katafuchi R, Yanase T, Ikeda K, Tanaka H, Fujimi S |title=Steroid responsiveness and frequency of relapse in adult-onset minimal change nephrotic syndrome |journal=Am. J. Kidney Dis. |volume=39 |issue=3 |pages=503–12 |date=March 2002 |pmid=11877569 |doi=10.1053/ajkd.2002.31400 |url=}}</ref><ref name="pmid8941578">{{cite journal |vauthors=Mak SK, Short CD, Mallick NP |title=Long-term outcome of adult-onset minimal-change nephropathy |journal=Nephrol. Dial. Transplant. |volume=11 |issue=11 |pages=2192–201 |date=November 1996 |pmid=8941578 |doi= |url=}}</ref> | * Adults who are positive with [[minimal change disease]] present with [[edema]] and most commonly with [[hypertension]].<ref name="pmid11877569">{{cite journal |vauthors=Nakayama M, Katafuchi R, Yanase T, Ikeda K, Tanaka H, Fujimi S |title=Steroid responsiveness and frequency of relapse in adult-onset minimal change nephrotic syndrome |journal=Am. J. Kidney Dis. |volume=39 |issue=3 |pages=503–12 |date=March 2002 |pmid=11877569 |doi=10.1053/ajkd.2002.31400 |url=}}</ref><ref name="pmid8941578">{{cite journal |vauthors=Mak SK, Short CD, Mallick NP |title=Long-term outcome of adult-onset minimal-change nephropathy |journal=Nephrol. Dial. Transplant. |volume=11 |issue=11 |pages=2192–201 |date=November 1996 |pmid=8941578 |doi= |url=}}</ref> | ||
* First-line therapy in adults with minimal change disease low-sodium diet and diuretics for fluid removal. | * First-line [[therapy]] in adults with [[minimal change disease]] low-[[sodium]] diet and [[Diuretic|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 | * [[Angiotensin-converting enzyme]] (ACE) inhibitors or [[Angiotensin II receptor antagonist|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.<ref name="pmid18580865">{{cite journal |vauthors=Galle J |title=Reduction of proteinuria with angiotensin receptor blockers |journal=Nat Clin Pract Cardiovasc Med |volume=5 Suppl 1 |issue= |pages=S36–43 |date=July 2008 |pmid=18580865 |doi=10.1038/ncpcardio0806 |url=}}</ref> | ||
==== Non Immunosuppressive therapies ==== | ==== Non Immunosuppressive therapies ==== | ||
* Glucocorticoid therapy with | * [[Glucocorticoid]] therapy with [[prednisone]] or [[prednisolone]] in [[minimal change disease]](MCD) patients.<ref name="pmid19494796">{{cite journal |vauthors=Meyrier AY |title=Treatment of focal segmental glomerulosclerosis with immunophilin modulation: when did we stop thinking about pathogenesis? |journal=Kidney Int. |volume=76 |issue=5 |pages=487–91 |date=September 2009 |pmid=19494796 |doi=10.1038/ki.2009.204 |url=}}</ref><ref name="pmid23431071">{{cite journal |vauthors=Hogan J, Radhakrishnan J |title=The treatment of minimal change disease in adults |journal=J. Am. Soc. Nephrol. |volume=24 |issue=5 |pages=702–11 |date=April 2013 |pmid=23431071 |doi=10.1681/ASN.2012070734 |url=}}</ref><ref name="pmid3747335">{{cite journal |vauthors=Nolasco F, Cameron JS, Heywood EF, Hicks J, Ogg C, Williams DG |title=Adult-onset minimal change nephrotic syndrome: a long-term follow-up |journal=Kidney Int. |volume=29 |issue=6 |pages=1215–23 |date=June 1986 |pmid=3747335 |doi= |url=}}</ref> | ||
* Glucocorticoid have antiproteinuric effect on the glomerular filtration barrier apart from the immunosuppressive effect.<ref name="pmid4916790">{{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> | * [[Glucocorticoid]] have antiproteinuric effect on the [[glomerular filtration]] barrier apart from the [[Immunosuppression|immunosuppressive]] effect.<ref name="pmid4916790">{{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> | ||
** Preferred regimen (1): | ** 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.<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> | ||
==References== | ==References== | ||
Revision as of 15:21, 8 June 2018
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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.
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]
- 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.
- 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 (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]
Initial therapy for adults
- Adults who are positive with minimal change disease present with edema and most commonly with hypertension.[3][4]
- 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.[5]
Non Immunosuppressive therapies
- Glucocorticoid therapy with prednisone or prednisolone in minimal change disease(MCD) patients.[6][7][8]
- Glucocorticoid have antiproteinuric effect on the glomerular filtration barrier apart from the immunosuppressive effect.[9]
- 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.[10]
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.
- ↑ 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.