Familial Mediterranean Fever (Recurrent Hereditary Polyserositis) in Arabs - A Study of 175 Patients and Review of the Literature

Abstract

Recurrent hereditary polyserositis (RHP) or familial Mediterranean fever (FMF) is a chronic inherited illness of obscure aetiology. The disease is characterised by paroxysmal attacks of fever, peritonitis, pleuritis or arthritis, and predominantly affects Sephardic Jews, Arabs, Turks and Armenians. In this study, we report our 11-year experience of 175 Arab patients with this disease. As with other ethnic groups, the most common manifestation (93.7 per cent) was peritonitis. Arthritis (33.7 per cent) and pleurisy (32 per cent) were next in frequency. Adult patients in this series unlike those in other ethnic groups, rarely presented with arthritis. Similarly rare were amyloidosis, rashes, splenomegaly, hepatomegaly or lymphadenopathy. The aetiology of this disease is not clear but we suspect that abnormalities in catecholamine metabolism may be a factor in the pathogenesis.