Goodpasture syndrome medical therapy

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.

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.

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.

↑ 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.