Current therapeutic options for managing familial Mediterranean fever

Introduction: Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterized by recurrent attacks of fever and serositis. Although colchicine prevents the recurrence of FMF attacks and the development of secondary amyloidosis, the major long-term complication of FMF in most of the patients; 5 - 10% of patients may have inadequate symptom control despite good adherence to colchicine in maximally tolerated doses. Areas covered: In this review, we summarize the treatment approaches for FMF patients to prevent and treat acute attacks, control chronic inflammation, and treat special manifestations and complications. Expert opinion: Colchicine remains the gold standard treatment of FMF. Although colchicine prevents the recurrence of FMF attacks and the development of secondary amyloidosis, 5 - 10% of patients do not respond to conventional colchicine treatment in maximally tolerated doses. Based on the findings of hypersecretion of IL-1 beta in auto-inflammatory diseases as FMF, IL-1 inhibitors have been proposed as a treatment for crFMF. Attack treatment is mainly supportive and biologic drugs such as anti-IL-1 and anti-IL-6 are added to colchicine therapy in secondary amyloidosis.