Goodpasture syndrome medical therapy
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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.