Familial Mediterranean Fever

Familial Mediterranean fever (FMF) is a recessively inherited chronic inflammatory disease characterized by recurrent episodes of febrile sterile polyserositis. It is associated with a mutation in the MEFV gene, encoding pyrin, an immune-regulatory protein. It has been shown repetitively that in chronic inflammatory diseases, the inflammatory burden is associated with an increased rate of atherosclerosis. Nevertheless, the association between FMF, a paradigm for inflammation, and increased rate of ischemic heart disease is doubtful. Moreover, some studies have suggested the presence of electrocardiographic markers, implying an increased risk in FMF for developing ventricular or supraventricular arrhythmias. However these findings are also debatable due to other studies with conflicting results and the lack of any evidence for increased rate of arrhythmias. Another mode of cardiac involvement in FMF is inflammation of the pericardium, which may rarely be complicated by large pericardial effusion and constrictive pericarditis. Finally, reactive amyloidosis, which is the most devastating complication of FMF, and appears almost exclusively in colchicine untreated patients, may on rare occasions involve the heart, mainly with diastolic dysfunction. Herein, we review all published data on predictors and actual findings of cardiac disease in FMF and discuss the cardiovascular effects of the mutated MEFV gene.