Background Familial mediterranean fever (FMF) is an inherited multisystem disease of unknown aetiology, characterised by recurrent, painful, self-limited episodes.
Objectives The objectives of this study is to describe clinical profile, course and complications of 45 cases of FMF observed over a period of 25 years.
Methods Retrospective review of 45 cases with FMF.
Results Forty patients (88%) started their illness below the age of 10 years. Peritonitis occured in 84,4%, arthritis in 35,5%, pleuretis in 17,7% and erysipelas-like lesions in 31,1%. 21 patients developed renal amyloïdosis and 17 patients were subjected to unnecessary operative surgery.
The arthritis was monoarticular in 85% and polyarticular in 15% and was seronegative in all patients (Rheumatoïd factor and antinuclear antibodies). The synovial attack showed a wide variation in the clinical presentation, course and duration of arthritis causing diagnostic difficulties. Renal amyloïdosis occurred in 21 patients after a mean duration of FMF 10,5 years (2 to 28 years). One patient had hematuria, kidney biopsy showed mesangial IgA glomerulonephritis. Among 32 patients treated with colchicine, 80% achieved a therapeutic response.
Conclusion FMF is an inherited disease; colchicine remains the only efficient treatment to prevent both acute manifestations and amyloïdosis.
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