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AB0221 Familial mediterranean fever: analysis of 45 tunisian patients
  1. SS Turki,
  2. CH Ben Taarit,
  3. R Goucha,
  4. E Abderrahim,
  5. F Ben Moussa,
  6. H Ben Maiz
  1. Department of Nephrology and Internal Medicine, Charles Nicolle Hospital, Tunis, Tunisia

Abstract

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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