Familial Mediterranean fever

Familial Mediterranean fever

Familial Mediterranean fever (FMF; synonym familial paroxysmal polyserositis) is an autosomal recessive inherited autoinflammatory syndrome characterised by episodic di ff use abdominal pain and tenderness, mild pyrexia and joint pain. - Symptoms are usually mild and resolve within 24–72 hours. Rarely pericardial or meningeal inflammation may occur. Amyloidosis is a long-term complication. FMF is associated with mutations in the MEFV (Mediterra - nean fever) gene most frequently found in Arab, Armenian and - Jewish populations. MEFV encodes the protein pyrin, which is expressed in neutrophils and is thought to regulate interleu - - kin-1B (a proinflammatory cytokine) release. Symptoms often present in childhood and may be misdiag - nosed as appendicitis. Treatment of an acute episode is symp - tomatic. Colchicine can be used to reduce the frequency and severity of attacks and to prevent development of amyloidosis .

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