C5a-Inhibitor Deficiency — A Role in Familial Mediterranean Fever?

The sequence of events that precipitate the recurrent febrile attacks of familial Mediterranean fever has fascinated clinicians ever since the disease became firmly established as a distinct clinical entity in 1945.1 Clues to its cause seemed to be provided by its prevalence in specific ethnic groups in the Mediterranean littoral, a high familial incidence, and the clinical manifestations of self-limited, febrile paroxysms and serosal inflammation. Early authors, impressed by the intermittency of attacks and a high incidence of atopy, searched vainly for an exogenous allergen. In a comprehensive study of the disease in Israel in 1958, Heller at al. postulated . . .