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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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Detalles Bibliográficos
Autores principales: Malho, Anabela, Santos, Viriato, Cabrita, Ana, Silva, Ana Paula, Pinto, Isabel, Bernardo, Idalécio, Neves, Pedro Leão
Formato: Texto
Lenguaje:English
Publicado: SAGE-Hindawi Access to Research 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2989737/
https://www.ncbi.nlm.nih.gov/pubmed/21152201
http://dx.doi.org/10.4061/2010/383548
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author Malho, Anabela
Santos, Viriato
Cabrita, Ana
Silva, Ana Paula
Pinto, Isabel
Bernardo, Idalécio
Neves, Pedro Leão
author_facet Malho, Anabela
Santos, Viriato
Cabrita, Ana
Silva, Ana Paula
Pinto, Isabel
Bernardo, Idalécio
Neves, Pedro Leão
author_sort Malho, Anabela
collection PubMed
description 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.
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spelling pubmed-29897372010-12-09 Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil Malho, Anabela Santos, Viriato Cabrita, Ana Silva, Ana Paula Pinto, Isabel Bernardo, Idalécio Neves, Pedro Leão Int J Nephrol Case Report 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. SAGE-Hindawi Access to Research 2010-08-16 /pmc/articles/PMC2989737/ /pubmed/21152201 http://dx.doi.org/10.4061/2010/383548 Text en Copyright © 2010 Anabela Malho et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Malho, Anabela
Santos, Viriato
Cabrita, Ana
Silva, Ana Paula
Pinto, Isabel
Bernardo, Idalécio
Neves, Pedro Leão
Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil
title Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil
title_full Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil
title_fullStr Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil
title_full_unstemmed Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil
title_short Severe Relapsing Goodpasture's Disease Successfully Treated with Mycophenolate Mofetil
title_sort severe relapsing goodpasture's disease successfully treated with mycophenolate mofetil
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2989737/
https://www.ncbi.nlm.nih.gov/pubmed/21152201
http://dx.doi.org/10.4061/2010/383548
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