Current therapeutic options for managing familial Mediterranean fever

Abstract

Introduction: Familial Mediterranean fever (FMF) is a monogenic auto-inflammatory 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-1β 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.

Keywords:

• amyloidosis
• anti-IL-1
• colchicine
• familial Mediterranean fever
• treatment

Declaration of interest

S Ozen is receiving consultancy and speaker fees from Novartis and Sobi. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Notes

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