The clinical role of anakinra in the armamentarium against familial Mediterranean fever

ABSTRACT

Introduction

Familial Mediterranean Fever (FMF) is the most common autoinflammatory disease that has mainly been treated with colchicine since 1972. A significant portion of patients do not respond to colchicine and require further treatment, mainly IL-1β antagonists such as anakinra, canakinumab and rilonacept as IL-1β has a crucial role in pathogenesis of FMF. This review summarizes the current approach to treating FMF and discovers the pharmacological and clinical utility of IL-1 blocking agents based on accumulated evidence with a focus on anakinra.

Areas covered

This review focuses on anakinra treatment in FMF. The data obtained from case reports, case series, retrospective studies and a Phase III trial are analyzed. Safety and efficacy profiles of anakinra are discussed.

Expert opinion

Anakinra is the cheapest anti-IL-1 agent used in the treatment of colchicine-resistant FMF. It is shown to be effective and safe when used in adjunct to colchicine however its short half-life and potential to cause injection site reactions limit its use.

KEYWORDS:

• Anakinra
• familial Mediterranean fever
• hereditary periodic fever syndromes
• colchicine-resistant familial Mediterranean fever
• anti IL-1 drugs
• IL-1β

Article highlights

• Familial Mediterranean Fever (FMF) is the most common autoinflammatory disease. It can be complicated by AA amyloidosis if not treated properly. Colchicine has been the mainstay of treatment since 1972.

• The MEFV gene encodes pyrin/marenostrin protein. The pyrin protein is a regulator of inflammation mediated by IL-1a, which plays a major role in the pathogenesis of FMF.

• Anti-IL-1 agents are used to treat colchicine resistant FMF. There are three anti-IL-1 agents available: anakinra, canakinumab and rilonacept.

• Anakinra is the cheapest anti-IL-1 agent used in the treatment of FMF however its short half-life and potential to cause injection site reactions limit its use.

• The data obtained from case reports, case series, retrospective studies and a Phase III trial suggest that anakinra is safe and effective in treating colchicine resistant FMF.

• Current research efforts are tackling further prevention and treatment of amyloidosis and further improve the disease mortality.

Declaration of interest

The authors have no 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.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose

Additional information

Funding

This paper was not funded.