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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Bibliographic Details
Main Authors: Anabela Malho, Viriato Santos, Ana Cabrita, Ana Paula Silva, Isabel Pinto, Idalécio Bernardo, Pedro Leão Neves
Format: Article
Language:English
Published: Hindawi Limited 2010-01-01
Series:International Journal of Nephrology
Online Access:http://dx.doi.org/10.4061/2010/383548
Description
Summary: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