Secondary amyloidosis due to FMF

Abstract

Familial Mediterranean fever (FMF) is an ethnically restricted disease with an autosomal recessive inheritance characterized by recurrent attacks of fever, painful manifestations in the abdomen, chest and joints. The disease affects mainly non-Ashkenazi Jews, Armenians, Turks Arabs and other people of Mediterranean origin. The disease may present at any age, more than 80% of patients being symptomatic by the age of 20. Although the inflammatory attacks that characterize the disease may sometimes be debilitating, secondary (AA) amyloidosis remains the most serious manifestation of FMF causing considerable morbidity due mostly to nephropathic amyloidosis. The largest series of secondary amyloidosis in FMF have been reported from Turkey. The pathophysiological steps in progressing a patient from FMF to amyloidosis are not definitely known. Daily treatment with colchicine can prevent both the attacks and amyloid deposition but no effective alternative treatment exists for colchicine resistant cases. Meanwhile more population based epidemiological and genetic data should be gathered by worldwide collaborative studies to elucidate the link between FMF and amyloidosis and to develop alternative therapies.