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Abstract

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Article in Japanese

Case Report

Goodpasture syndrome: Eleven years of remission resulting in a relapsed alveolar hemorrhage

Naohito Suzuki  Haruhiko Nakajima 

Division of Respiratory Medicine, Ageo Central General Hospital

ABSTRACT

This report examines a unique case of Goodpasture syndrome, an anti-glomerular basement membrane (anti-GBM) antibody disease, found in a male patient. At 60 years of age, the patient was admitted to a hospital because of massive hemoptysis. His illness was initially diagnosed as “idiopathic alveolar hemorrhage,” serum with a negative anti-GBM antibody, and recovered through steroid pulse therapy and plasmapheresis. Steroids were then tapered and ultimately stopped when he turned 63, and he continued follow-up appointments with a general physician. At 71, the patient was taken by ambulance to our hospital in an emergency with both massive hemoptysis and dyspnea. He also showed severe anemia and severe renal failure. Serum tests demonstrated that the anti-GBM antibody was positive, ANCA was negative, and ANA was also negative. He was intubated and underwent mechanical ventilation, steroid pulse therapy, hemodialysis, and transfusion on site. Nevertheless, he died seven days after being admitted. The autopsy revealed a diffused alveolar hemorrhage with neutrophilic alveolitis and vasculitis, and crescentic glomerulonephritis. This case suggests that the diffused alveolar hemorrhage may have been a relapse even after an extended period of remission. Where the patient suffered from an alveolar hemorrhage, the case stands as an example warranting long-term follow-up by a pulmonologist in all future cases of alveolar hemorrhaging.

KEYWORDS

Goodpasture syndrome  Alveolar hemorrhage  Anti-GBM antibody  Glomerulonephritis  Relapse 

Received 26 May 2016 / Accepted 12 Sep 2016

AJRS, 6(1): 22-26, 2017

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