pediatrics
Familial Mediterranean Fever
Introduction
Familial Mediterranean fever is an autosomal recessive disorder characterized by recurrent bouts of fever and peritonitis, sometimes with pleuritis, erysipelas-like erythema, arthritis, and, rarely, pericarditis. Amyloidosis may develop, sometimes leading to renal failure. People with genetic origins in the Mediterranean basin are more frequently affected than other ethnic groups. Diagnosis is based on clinical suspicion and genetic testing. Treatment with prophylacticcolchicineprevents acute attacks as well as amyloidosis in almost all patients. Patients who arecolchicine-resistant or -intolerant may be treated with interleukin-1 inhibitors (anakinra,canakinumab,rilonacept). Prognosis is excellent with treatment. Familial Mediterranean fever (FMF) is a disease of people with genetic origins in the Mediterranean basin, predominantly people who have Sephardic Jewish, North African Arab, Armenian, Turkish, Greek, or Italian ancestry. However, cases have occurred among enough other groups (eg, people who have Ashkenazi Jewish, Cuban, or Japanese ancestry), so the diagnosis should not be excluded solely on the basis of ancestry. 