Abstract
Goodpasture's syndrome in association with silicosis in a 36-year-old male foundry worker was presented.
Three months after the development of initial symptoms including fever, anaemia, purpura, polyarthralgia, shortness of breath and Raynaud's phenomenon in winter, the patient was hospitalized.
Silicosis, mild renal insufficiency and iron deficiency anaemia were found. Renal biopsy revealed focal embolic glomerulonephritis. Rumpel-Leede test was positive, ESR increased and CRP strongly positive; hypergammaglobulinaemia and strongly positive RA reactions were noticed.
After admission, renal insufficiency and anaemia progressed rapidly with development of generalised oedema, retinal bleeding and eruptions resembling multiple exudative erythema. Corticosteroid administration and blood transfusion induced a remission lasting for 5 months.
Ten months after the onset of symptoms, he died following sudden aggravation of dyspnoea with haemoptysis. Autopsy revealed, in addition to silicosis, diffuse haemorrhage of the lungs and diffuse subacute glomerulonephritis, compatible with Goodpasture's syndrome.
Silicosis probably bears a causal relationship to Goodpasture's syndrome in this case through an autoimmune mechanism. Another case of Goodpasture's syndrome associated with silicosis is found in the literature (Burilkov, T. et al.).
The initial symptoms suggest the possibility of intrapulmonary bleeding occurring long before the onset of clinical manifestations, although haemoptysis was a terminal event.
The skin rash seen in this case had already been described in Osler's cases preceding Goodpasture's original report.
Concerning the remission from advanced renal failure, Munro, J. B. et al. reported a similar result.
The present case seems to suggest that oedema, retinal bleeding, purpura and eruptions resembling multiple exudative erythema are indications of poor prognosis.
It is the 10th case of Goodpasture's syndrome reported in Japan and the 6th autopsy-confirmed case in the literature.