Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil

Goodpasture's disease is a rare autoimmune disorder characterised by the development of antiglomerular basement membrane autoantibodies, which typically presents with rapidly progressive crescentic glomerulonephritis and pulmonary haemorrhage. Even with aggressive nonspecific immunosuppression...

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Veröffentlicht in:International Journal of Nephrology 2010-01, Vol.2010 (2010), p.21-23
Hauptverfasser: Guedes, Anabela Malho, Santos, Viriato, Vieira, Ana de Lurdes Agostinho Cabrita, Silva, Ana Paula, Pinto, Isabel, Bernardo, Idalécio, Neves, Pedro Leão
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Sprache:eng
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Zusammenfassung:Goodpasture's disease is a rare autoimmune disorder characterised by the development of antiglomerular basement membrane autoantibodies, which typically presents with rapidly progressive crescentic glomerulonephritis and pulmonary haemorrhage. Even with aggressive nonspecific immunosuppression and plasma exchange, mortality remains high. We report a case of life-threatening Goodpasture's disease with relapsing pulmonary haemorrhage refractory to conventional therapy. Second line treatment was based on mycophenolate mofetil 1 g every 12 hours and prednisolone 60 mg/day. In this case, the use of a low-dose mycophenolate mofetil regimen turned out to be insufficient. However, in our opinion higher mycophenolate mofetil doses should be considered an alternative treatment, mainly in relapsing disease, due to its mechanism of action and current insufficient therapeutic weapons.
ISSN:2090-2158
2090-214X
2090-2158
DOI:10.4061/2010/383548