Objective. To establish a new set of criteria for the diagnosis of familial Mediterranean fever (FMF).Methods. Twenty-seven features and manifestations typical of FMF were studied to determine their prevalence in 105 patients with FMF and 106 controls. Diagnosis of FMF in the study group was based on clinical judgment. Controls were patients with a variety of other diseases who presented to the emergency room or outpatient clinics with recurrent episodes of pain in body sites usually involved in FMF attacks. Manifestations observed to be significantly more common in FMF patients than in controls were incorporated into the rule proposed for diagnosis of FMF, based on a model of major, minor, and supportive criteria.Results. Two sets of diagnostic criteria were established. A conservative criteria set for diagnosis of FMF was based on the presence of 1 major or 2 minor criteria, or 1 minor plus 5 supportive criteria, and a simple criteria set for diagnosis of FMF required 1 major or 2 minor criteria. The sensitivity and specificity of these 2 criteria sets were >95% and >97%, respectively.Conclusion. The proposed new sets of criteria were highly sensitive and specific, and could be used to readily diagnose FMF and to distinguish FMF from other periodic febrile diseases.Familial Mediterranean fever (FMF) is a genetic disease that affects Sephardic Jews, Arabs, Turks, and Armenians, and is characterized by bouts of fever and

Familial Mediterranean fever (FMF) is characterized by recurrent attacks of febrile serositis. While arthritis, pleuritis and peritonitis are common in FMF, no association of pericarditis with FMF has been described in detail. We retrospectively studied about 4000 FMF patients, using a computer chart review. Pericarditis was diagnosed when patients sustained attacks of pleuritic retrosternal chest pain and had typical findings in the electrocardiogram, echocardiogram or chest radiogram. The incidence and features of pericarditis in FMF were compared to published data. Over a period of 20 years, one or more episodes of pericarditis were recorded in 27 patients, a significantly higher incidence than in the general population (68 vs. 6 per 10(5) per year, p < 0.001). Each patient experienced 1-3 pericarditis attacks, lasting a mean of 4.2 days, accompanied by high temperature and symptoms of FMF attack at another site. The pericarditis attack resolved spontaneously and left no sequelae. FMF patients with pericarditis were comparable to other FMF patients in most demographic and clinical parameters. Pericarditis may be considered another rare manifestation of FMF.

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