Терапевтический архив (Jun 2018)

Atypical Goodpasture’s disease: a clinical case report and literature review

  • M L Bulanova,
  • D V Potapov,
  • N M Bulanov,
  • L V Lysenko(Kozlovskaya)

DOI
https://doi.org/10.26442/terarkh2018906130-136
Journal volume & issue
Vol. 90, no. 6
pp. 130 – 136

Abstract

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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.

Keywords