The Use of IL-1 Receptor Antagonist (Anakinra) in Idiopathic Recurrent Pericarditis: A Narrative Review

Abstract

Recurrent pericarditis is a complication of acute pericarditis in 20-30% of the patients and is usually idiopathic in nature. The underlying pathogenesis of this condition remains unclear, although immune-mediated mechanisms seem likely. A subgroup of these patients with refractory symptoms can be challenging to manage, and multiple immunosuppressive medications have been used without consistent benefit. Anakinra, an interleukin-1 receptor antagonist, has been used in treatment of rheumatoid arthritis and autoinflammatory syndromes. Preliminary evidence suggests that anakinra could be a promising therapy for idiopathic recurrent pericarditis. In this narrative review, we summarize the current understanding of the etiopathogenesis of idiopathic recurrent pericarditis, mechanism of action of anakinra, and the preliminary evidence, supporting the use of anakinra in pericarditis.

Figure 1

Mechanism of action of anakinra. Both IL-1α and IL-1β act through IL-1 receptor 1 to stimulate the production of inflammatory cytokines and TNFα that lead to the inflammatory cascade. The inflammasome is a complex of distinct proteins which together convert inactive prointerleukin-1β to active IL-1β. Environmental and infectious triggers can mediate the formation of the inflammasome. Anakinra blocks IL-1 receptor 1, antagonizing the effects of both IL-1α and IL-1β. ASC: Apoptosis associated speck-like protein containing caspase activation and recruitment domain, IL: interleukin, IL-1-R1: interleukin-1 receptor 1, NF-κB: nuclear factor kappa-light-chain-enhancer of activated B cells, NLRP3: NOD-Like Receptor containing pyrin domain 3.