Atypical Goodpasture’s disease: a clinical case report and literature review
Goodpasture’s disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomeruloneph...
Main Authors: | , , , |
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Format: | Article |
Language: | Russian |
Published: |
"Consilium Medicum" Publishing house
2018-06-01
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Series: | Терапевтический архив |
Subjects: | |
Online Access: | https://ter-arkhiv.ru/0040-3660/article/viewFile/32816/pdf |
Summary: | Goodpasture’s disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomerulonephritis and alveolar hemorrhage (pulmonary-renal syndrome). We present a case of atypical disease course in a young male patient who developed alveolar hemorrhage without renal failure. The only symptom of renal involvement was isolated hematuria. Plasmapheresis combined with immunosuppression (cyclophosphamide and corticosteroids) was effective. We present a review of state-of-art data on the pathogenesis and disease course of anti-GBM disease. |
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ISSN: | 0040-3660 2309-5342 |