Familial Mediterranean Fever in an Italian Family

Abstract

In recent years the syndrome of familial Mediterranean fever (FMF) has been recognized as a distinct clinical entity,^1,2and a close relationship of the disease to amyloidosis has been established.³Familial 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.⁴On occasion a similar syndrome has been encountered in members of other ethnic groups (French, Spanish, Turkish, Greek, Swedish, and Dutch)³in 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 patients³and