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February 1970

Familial Mediterranean Fever in an Italian Family

Author Affiliations

New York

From the Department of Medicine, Rheumatic Diseases Study Group, New York University Medical Center, New York.

Arch Intern Med. 1970;125(2):337-340. doi:10.1001/archinte.1970.00310020143022

In recent years the syndrome of familial Mediterranean fever (FMF) has been recognized as a distinct clinical entity,1,2 and a close relationship of the disease to amyloidosis has been established.3 Familial Mediterranean fever is an inherited disease, most often seen in Sephardic Jews, Armenians, and Arabs, in whom it appears to be transmitted by an autosomal-recessive mode of inheritance.4 On occasion a similar syndrome has been encountered in members of other ethnic groups (French, Spanish, Turkish, Greek, Swedish, and Dutch) 3 in whom it occasionally appears to have been transmitted in a way compatible with an autosomal-dominant form of inheritance.

Familial Mediterranean fever has been divided into two phenotypes. Phenotype 1, the most commonly described variant, is characterized by recurrent attacks of fever and short self-limited peritonitis, pleurisy, or arthritis. Perireticular amyloidosis may occur during the course of the disease in about 26.5% of patients 3 and

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