Focal segmental glomerulosclerosis medical therapy: Difference between revisions
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{{Focal segmental glomerulosclerosis}} | {{Focal segmental glomerulosclerosis}} | ||
{{CMG}} | {{CMG}} | ||
==Overview== | ==Overview== | ||
Randomized clinical trials have only addressed patients with primary FSGS and have nephrotic-range proteinuria. For those patients, the use of corticosteroids and immunosuppressive therapy followed by conserative therapy with ACE-I or ARBs is recommended. For patients with relapse, guidelines for relapsing minimal change disease are recommended for patients with FSGS. Finally, patients with resistance to steroids are recommended to use cyclosporine, or a combination of mycophenolate mofetil and high dose dexamethasone for patients who cannot tolerate cyclosporine. | |||
==Medical Therapy== | ==Medical Therapy== | ||
* | The use of medical therapy in focal segmental glomerulosclerosis (FSGS) is based on the Kidney Disease - Improve Global Outcomes (KDIGO) guidelines in 2012. | ||
* | |||
===Initial Treatment<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>=== | |||
The use of both corticosteroids and immunosuppressive therapy is recommended for initial treatment in patients with idiopathic FSGS and who have nephrotic syndrome. For patients with secondary FSGS or non-nephrotic range proteinuria, there are currently no reliable clinical trials to support the use of steroids.(180) | |||
* | ====Corticosteroids==== | ||
Either prednisone or prednisolone may be used. Both have equivalent dosage and duration. | |||
*''Dose'': Single dose of 1 mg/kg (maximum 80 mg) or alternate-day dose of 2 mg/kg (maximum 120 mg). | |||
*''Duration'': Minimum 4 weeks - maximum 16 weeks, as tolerated, or until complete remission is achieved. | |||
Steroids should be tapered slowly over a period of 6 months after achieving complete remission. Patients with uncontrolled diabetes mellitus, psychiatric conditions, and severe osteoporosis have a relative contraindication to the use of steroids and may benefit more from calcineurin inhibitors (CNI). | |||
In patients with tip lesions, good renal function, and low degrees of proteinuria, the time for initiation of corticosteroids is controversial, and may occur following the use of renin-angiotensin-system (RAS) blockers, such as ACE-inhibitors or ARBs, to assess for possible spontaneous remission. | |||
====Calcineurin Inhibitors (CNI)<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>==== | |||
For patients with relative contraindication to steroids, such as patients with uncontrolled diabetes mellitus, psychiatric conditions, or severe osteoporosis, the use of CNI might be more helpful. | |||
===Conservative Treatment<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>=== | |||
Renin-angiotensin-system (RAS) blockade is also necessary for patients with proteinuria, including those with FSGS. The initiation of RAS blockade may take place following initial corticosteroid therapy in patients with nephrotic syndrome to assess for remission on steroids, which is especially implicated in patients with severe nephrotic syndrome. | |||
===Treatment for Relapse<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>=== | |||
Treatment for relapse is based upon the guidelines for the management of relapse of minimal change disease in adults. | |||
===Treatment for Steroid-Resistant FSGS=== | |||
====Cyclosporine<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>==== | |||
*''Dose'':3-5 mg/kg/d in divided doses | |||
*''Duration'': At least 4-6 months. Cyclosporine therapy is to be continued for at least 12 months only if there is partial or complete remission with use. | |||
Slow tapering of cyclosporine should be achieved following the initial duration of use. For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil and high-dose dexamethasone is recommended. | |||
====Other==== | |||
For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil (MMF) and high-dose dexamethasone is recommended.<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> | |||
==References== | ==References== |
Revision as of 01:06, 4 December 2013
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Randomized clinical trials have only addressed patients with primary FSGS and have nephrotic-range proteinuria. For those patients, the use of corticosteroids and immunosuppressive therapy followed by conserative therapy with ACE-I or ARBs is recommended. For patients with relapse, guidelines for relapsing minimal change disease are recommended for patients with FSGS. Finally, patients with resistance to steroids are recommended to use cyclosporine, or a combination of mycophenolate mofetil and high dose dexamethasone for patients who cannot tolerate cyclosporine.
Medical Therapy
The use of medical therapy in focal segmental glomerulosclerosis (FSGS) is based on the Kidney Disease - Improve Global Outcomes (KDIGO) guidelines in 2012.
Initial Treatment[1]
The use of both corticosteroids and immunosuppressive therapy is recommended for initial treatment in patients with idiopathic FSGS and who have nephrotic syndrome. For patients with secondary FSGS or non-nephrotic range proteinuria, there are currently no reliable clinical trials to support the use of steroids.(180)
Corticosteroids
Either prednisone or prednisolone may be used. Both have equivalent dosage and duration.
- Dose: Single dose of 1 mg/kg (maximum 80 mg) or alternate-day dose of 2 mg/kg (maximum 120 mg).
- Duration: Minimum 4 weeks - maximum 16 weeks, as tolerated, or until complete remission is achieved.
Steroids should be tapered slowly over a period of 6 months after achieving complete remission. Patients with uncontrolled diabetes mellitus, psychiatric conditions, and severe osteoporosis have a relative contraindication to the use of steroids and may benefit more from calcineurin inhibitors (CNI).
In patients with tip lesions, good renal function, and low degrees of proteinuria, the time for initiation of corticosteroids is controversial, and may occur following the use of renin-angiotensin-system (RAS) blockers, such as ACE-inhibitors or ARBs, to assess for possible spontaneous remission.
Calcineurin Inhibitors (CNI)[1]
For patients with relative contraindication to steroids, such as patients with uncontrolled diabetes mellitus, psychiatric conditions, or severe osteoporosis, the use of CNI might be more helpful.
Conservative Treatment[1]
Renin-angiotensin-system (RAS) blockade is also necessary for patients with proteinuria, including those with FSGS. The initiation of RAS blockade may take place following initial corticosteroid therapy in patients with nephrotic syndrome to assess for remission on steroids, which is especially implicated in patients with severe nephrotic syndrome.
Treatment for Relapse[1]
Treatment for relapse is based upon the guidelines for the management of relapse of minimal change disease in adults.
Treatment for Steroid-Resistant FSGS
Cyclosporine[1]
- Dose:3-5 mg/kg/d in divided doses
- Duration: At least 4-6 months. Cyclosporine therapy is to be continued for at least 12 months only if there is partial or complete remission with use.
Slow tapering of cyclosporine should be achieved following the initial duration of use. For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil and high-dose dexamethasone is recommended.
Other
For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil (MMF) and high-dose dexamethasone is recommended.[1]