Goodpasture syndrome medical therapy: Difference between revisions
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{{CMG}}{{APM}}{{AE}}{{KW}} | {{CMG}}{{APM}}{{AE}}{{KW}} | ||
==Overview== | ==Overview== | ||
Currently there is no cure for Goodpasture syndrome.The mainstay of therapy for Goodpasture syndrome consist of | Currently there is no cure for Goodpasture syndrome. The mainstay of therapy for Goodpasture syndrome consist of [[corticosteroids]], [[cyclophosphamide]] and [[plasmapheresis]]. | ||
==Medical Therapy== | ==Medical Therapy== | ||
* The mainstay of treatment for Goodpasture’s syndrome includes medical therapy with [[corticosteroid]]s [[immunosuppressant]]s, and [[plasmapheresis]].<ref name="pmid25462583">{{cite journal| author=Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G et al.| title=Goodpasture's syndrome: a clinical update. | journal=Autoimmun Rev | year= 2015 | volume= 14 | issue= 3 | pages= 246-53 | pmid=25462583 | doi=10.1016/j.autrev.2014.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25462583 }}</ref><ref name="pmid11388816">{{cite journal |vauthors=Levy JB, Turner AN, Rees AJ, Pusey CD |title=Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression |journal=Ann. Intern. Med. |volume=134 |issue=11 |pages=1033–42 |date=June 2001 |pmid=11388816 |doi= |url=}}</ref><ref name="pmid25086644">{{cite journal |vauthors=Zhang YY, Tang Z, Chen DM, Gong DH, Ji DX, Liu ZH |title=Comparison of double filtration plasmapheresis with immunoadsorption therapy in patients with anti-glomerular basement membrane nephritis |journal=BMC Nephrol |volume=15 |issue= |pages=128 |date=August 2014 |pmid=25086644 |pmc=4127070 |doi=10.1186/1471-2369-15-128 |url=}}</ref> | * The mainstay of treatment for Goodpasture’s syndrome includes medical therapy with [[corticosteroid]]s [[immunosuppressant]]s, and [[plasmapheresis]].<ref name="pmid25462583">{{cite journal| author=Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G et al.| title=Goodpasture's syndrome: a clinical update. | journal=Autoimmun Rev | year= 2015 | volume= 14 | issue= 3 | pages= 246-53 | pmid=25462583 | doi=10.1016/j.autrev.2014.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25462583 }}</ref><ref name="pmid11388816">{{cite journal |vauthors=Levy JB, Turner AN, Rees AJ, Pusey CD |title=Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression |journal=Ann. Intern. Med. |volume=134 |issue=11 |pages=1033–42 |date=June 2001 |pmid=11388816 |doi= |url=}}</ref><ref name="pmid25086644">{{cite journal |vauthors=Zhang YY, Tang Z, Chen DM, Gong DH, Ji DX, Liu ZH |title=Comparison of double filtration plasmapheresis with immunoadsorption therapy in patients with anti-glomerular basement membrane nephritis |journal=BMC Nephrol |volume=15 |issue= |pages=128 |date=August 2014 |pmid=25086644 |pmc=4127070 |doi=10.1186/1471-2369-15-128 |url=}}</ref> | ||
* Treatment of Goodpasture syndrome should begin before serum creatinine levels are greater than 5.7mg/dl, before the development of anuria, and the need for renal dialysis. | * Treatment of Goodpasture syndrome should begin before [[serum creatinine]] levels are greater than 5.7mg/dl, before the development of [[anuria]], and the need for [[renal dialysis]]. | ||
* The normal duration of medical therapy in patients with Goodpasture syndrome includes:<ref name="pmid25018939">{{cite journal| author=| title=Chapter 14: Anti-glomerular basement membrane antibody glomerulonephritis. | journal=Kidney Int Suppl (2011) | year= 2012 | volume= 2 | issue= 2 | pages= 240-242 | pmid=25018939 | doi=10.1038/kisup.2012.27 | pmc=4089639 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25018939 }} </ref> | * The normal duration of medical therapy in patients with Goodpasture syndrome includes:<ref name="pmid25018939">{{cite journal| author=| title=Chapter 14: Anti-glomerular basement membrane antibody glomerulonephritis. | journal=Kidney Int Suppl (2011) | year= 2012 | volume= 2 | issue= 2 | pages= 240-242 | pmid=25018939 | doi=10.1038/kisup.2012.27 | pmc=4089639 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25018939 }} </ref> | ||
** [[Corticosteriod]]s therapy for at least 6 months and in tapering doses with [[immunosuppressant]]s for 2 to 3 months. | ** [[Corticosteriod]]s therapy for at least 6 months and in tapering doses with [[immunosuppressant]]s for 2 to 3 months. | ||
** [[Plasmapheresis]] is given for 10 to 14 treatments. | ** [[Plasmapheresis]] is given for 10 to 14 treatments. | ||
* Patients with advanced progression of Goodpasture syndrome however, are not always compliant to treatments of plasmapheresis, corticosteroids, and [[immunosuppressants]]. | * Patients with advanced progression of Goodpasture syndrome however, are not always compliant to treatments of [[plasmapheresis]], [[corticosteroids]], and [[immunosuppressants]]. | ||
** [[Plasmapheresis]] may still be of benefit in patients who present with pulmonary hemorrhage.<ref name="pmid12815141">{{cite journal| author=Hudson BG, Tryggvason K, Sundaramoorthy M, Neilson EG| title=Alport's syndrome, Goodpasture's syndrome, and type IV collagen. | journal=N Engl J Med | year= 2003 | volume= 348 | issue= 25 | pages= 2543-56 | pmid=12815141 | doi=10.1056/NEJMra022296 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12815141 }}</ref> | ** [[Plasmapheresis]] may still be of benefit in patients who present with [[pulmonary hemorrhage]].<ref name="pmid12815141">{{cite journal| author=Hudson BG, Tryggvason K, Sundaramoorthy M, Neilson EG| title=Alport's syndrome, Goodpasture's syndrome, and type IV collagen. | journal=N Engl J Med | year= 2003 | volume= 348 | issue= 25 | pages= 2543-56 | pmid=12815141 | doi=10.1056/NEJMra022296 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12815141 }}</ref> | ||
** An alternative treatment used for Goodpasture's is [[rituximab]].<ref name="pmid27049372">{{cite journal| author=Couser WG| title=Pathogenesis and treatment of glomerulonephritis-an update. | journal=J Bras Nefrol | year= 2016 | volume= 38 | issue= 1 | pages= 107-22 | pmid=27049372 | doi=10.5935/0101-2800.20160016 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27049372 }} </ref> | ** An alternative treatment used for Goodpasture's is [[rituximab]].<ref name="pmid27049372">{{cite journal| author=Couser WG| title=Pathogenesis and treatment of glomerulonephritis-an update. | journal=J Bras Nefrol | year= 2016 | volume= 38 | issue= 1 | pages= 107-22 | pmid=27049372 | doi=10.5935/0101-2800.20160016 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27049372 }} </ref> | ||
*** The use of [[rituximab]] for 2 to 4 weeks is beneficial in the recovery of renal function in patients undergoing renal dialysis. | *** The use of [[rituximab]] for 2 to 4 weeks is beneficial in the recovery of [[renal function]] in patients undergoing [[renal dialysis]]. | ||
*** The use of [[rituximab]] as an induction therapy for Goodpasture's is not ideal because of its slow activation time. | *** The use of [[rituximab]] as an induction therapy for Goodpasture's is not ideal because of its slow activation time. | ||
====Dosage==== | ====Dosage==== | ||
* 1.1 '''Corticostreoids''' | * 1.1 '''Corticostreoids''' | ||
** Preferred regimen (1): Prednisone at 1-1.5 miligram/kg PO q24 hrs daily. | ** Preferred regimen (1): [[Prednisone]] at 1-1.5 miligram/kg PO q24 hrs daily. | ||
** Preferred regimen (2): Patients with life-threatening Goodpasture syndrome are treated with IV methylprednisolone at the rate of 1 g/day for 3 days followed by gradual tapering of dose. | ** Preferred regimen (2): Patients with life-threatening Goodpasture syndrome are treated with IV [[methylprednisolone]] at the rate of 1 g/day for 3 days followed by gradual tapering of dose. | ||
* 2.1 '''Immunosuppressants''' | * 2.1 '''Immunosuppressants''' | ||
** Preferred regimen (1): Cyclophosphamide at 2 mg/kg PO q 24 hrs daily. | ** Preferred regimen (1): [[Cyclophosphamide]] at 2 mg/kg PO q 24 hrs daily. | ||
** Preferred regimen (2): Patients with life-threatening Goodpasture syndrome are treated with IV cyclophosphamide at the rate of 1 g/m2. | ** Preferred regimen (2): Patients with life-threatening Goodpasture syndrome are treated with IV [[cyclophosphamide]] at the rate of 1 g/m2. | ||
** Alternative regimen (1): Azathioprine 1 mg/kg/day IV/PO initially in a single daily dose with dose not to exceed 2.5 mg/kg/day. | ** Alternative regimen (1): [[Azathioprine]] 1 mg/kg/day IV/PO initially in a single daily dose with dose not to exceed 2.5 mg/kg/day. | ||
* 3.1 '''Monoclonal antibody''' | * 3.1 '''Monoclonal antibody''' | ||
** Preferred regimen (1): Rituximab in the starting dose of 375 mg/m² IV qWeek for 4 weeks. | ** Preferred regimen (1): [[Rituximab]] in the starting dose of 375 mg/m² IV qWeek for 4 weeks. | ||
==References== | ==References== | ||
Latest revision as of 15:14, 1 May 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]
Overview
Currently there is no cure for Goodpasture syndrome. The mainstay of therapy for Goodpasture syndrome consist of corticosteroids, cyclophosphamide and plasmapheresis.
Medical Therapy
- The mainstay of treatment for Goodpasture’s syndrome includes medical therapy with corticosteroids immunosuppressants, and plasmapheresis.[1][2][3]
- Treatment of Goodpasture syndrome should begin before serum creatinine levels are greater than 5.7mg/dl, before the development of anuria, and the need for renal dialysis.
- The normal duration of medical therapy in patients with Goodpasture syndrome includes:[4]
- Corticosteriods therapy for at least 6 months and in tapering doses with immunosuppressants for 2 to 3 months.
- Plasmapheresis is given for 10 to 14 treatments.
- Patients with advanced progression of Goodpasture syndrome however, are not always compliant to treatments of plasmapheresis, corticosteroids, and immunosuppressants.
- Plasmapheresis may still be of benefit in patients who present with pulmonary hemorrhage.[5]
- An alternative treatment used for Goodpasture's is rituximab.[6]
- The use of rituximab for 2 to 4 weeks is beneficial in the recovery of renal function in patients undergoing renal dialysis.
- The use of rituximab as an induction therapy for Goodpasture's is not ideal because of its slow activation time.
Dosage
- 1.1 Corticostreoids
- Preferred regimen (1): Prednisone at 1-1.5 miligram/kg PO q24 hrs daily.
- Preferred regimen (2): Patients with life-threatening Goodpasture syndrome are treated with IV methylprednisolone at the rate of 1 g/day for 3 days followed by gradual tapering of dose.
- 2.1 Immunosuppressants
- Preferred regimen (1): Cyclophosphamide at 2 mg/kg PO q 24 hrs daily.
- Preferred regimen (2): Patients with life-threatening Goodpasture syndrome are treated with IV cyclophosphamide at the rate of 1 g/m2.
- Alternative regimen (1): Azathioprine 1 mg/kg/day IV/PO initially in a single daily dose with dose not to exceed 2.5 mg/kg/day.
- 3.1 Monoclonal antibody
- Preferred regimen (1): Rituximab in the starting dose of 375 mg/m² IV qWeek for 4 weeks.
References
- ↑ Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G; et al. (2015). "Goodpasture's syndrome: a clinical update". Autoimmun Rev. 14 (3): 246–53. doi:10.1016/j.autrev.2014.11.006. PMID 25462583.
- ↑ Levy JB, Turner AN, Rees AJ, Pusey CD (June 2001). "Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression". Ann. Intern. Med. 134 (11): 1033–42. PMID 11388816.
- ↑ Zhang YY, Tang Z, Chen DM, Gong DH, Ji DX, Liu ZH (August 2014). "Comparison of double filtration plasmapheresis with immunoadsorption therapy in patients with anti-glomerular basement membrane nephritis". BMC Nephrol. 15: 128. doi:10.1186/1471-2369-15-128. PMC 4127070. PMID 25086644.
- ↑ "Chapter 14: Anti-glomerular basement membrane antibody glomerulonephritis". Kidney Int Suppl (2011). 2 (2): 240–242. 2012. doi:10.1038/kisup.2012.27. PMC 4089639. PMID 25018939.
- ↑ Hudson BG, Tryggvason K, Sundaramoorthy M, Neilson EG (2003). "Alport's syndrome, Goodpasture's syndrome, and type IV collagen". N Engl J Med. 348 (25): 2543–56. doi:10.1056/NEJMra022296. PMID 12815141.
- ↑ Couser WG (2016). "Pathogenesis and treatment of glomerulonephritis-an update". J Bras Nefrol. 38 (1): 107–22. doi:10.5935/0101-2800.20160016. PMID 27049372.