May 4, 1963


JAMA. 1963;184(5):418. doi:10.1001/jama.1963.03700180144013

The pathogenesis of amyloidosis is obscure, in spite of thoroughly adequate clinical and pathological descriptions of this morbid state. The study of familial Mediterranean fever appears to be among the most promising approaches to understanding the mechanisms leading to amyloidosis. Familial Mediterranean fever (FMF) appears in individuals of Mediterranean ancestry. The disease is characterized by short attacks of fever usually accompanied by peritonitis, pleuritis, and arthritis. It was assumed at first that the amyloidosis in this febrile state was secondary in form. However, pathologists reported that no lesions known to be associated with secondary amyloidosis could be found. Moreover, there was no relationship between the number, duration, and severity of clinical attacks and the presence of amyloidosis. These observations support the conclusions of Heller et al1 that FMF is due to a biochemical inborn error of metabolism, and that "the amyloidosis of familial Mediterranean fever is not caused by

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