Familial Mediterranean fever

Abstract

Background: The pathogenesis of familial Mediterranean fever (FMF) is unknown, and since no specific laboratory test is yet available, the diagnosis of FMF remains clinical. The purpose of this study was to review clinical characteristics of patients with FMF.

Methods: A total of 96 patients with FMF were evaluated either retrospectively (for those diagnosed before 1997) or prospectively (for those after 1997).

Results: The records of 54 male and 42 female patients were studied. All patients were Turks. Family history was positive in 72 patients (75%). Involved site was peritoneum in 73 (76%), joints in 65 (68%), and pleura in 16 (17%). Febrile myalgia occurred in 3 patients (3%), and erysipelas-like skin lesions were observed in 2 (2%). Fever was found in 93 patients (97%). Reactive systemic (AA) amyloidosis was found in 38 patients (40%).

Conclusions: Diagnostic problems persist despite increased understanding of the pathogenesis of FMF. Amyloidosis, the most important complication of FMF, is often seen.