Mortality and prognosis related to the amyloidosis of Still's disease.

Abstract
Among 389 cases of Still''s disease followed up for 1-25 years (mean 10. 9), 16 cases of amyloidosis were diagnosed, giving an overall incidence of 4.1%). At 10 years from the onset of the disease in 209 patients, 9 (4. 3%) had developed amyloidosis. The mean duration of Still''s disease at the time of diagnosis of amyloidosis was 8. 2 years (range 1 - 23). Amyloidosis was suspected by the presence of proteinuria, hepatomegaly or splenomegaly, and was confirmed in the past by the intravenous Congo red test and more recently by renal or rectal biopsy. Of the 16 cases, 7 died from 4 months - 14 years (mean 8) after tiie diagnosis of amyloidosis. The remaining 9 are still alive, the mean duration of confirmed amyloidosis being 4 yr. (range 6 months - 10 yr.). No definite correlation was found between previous treatment with corticosteroids or Au and the subsequent formation of amyloid. As yet no satisfactory treatment for amyloidosis is available.
Keywords