Our experience of anti-interleukin 1 therapy

Abstract

Cryopyrin-associated periodic syndromes (CAPS) are characterized by apparently unprovoked attacks of fever, rashes, and musculoskeletal and sensorineural inflammation accompanied by high acute-phase reactants. Excessive interleukin-1 (IL-1) signaling appears to be a constant feature in the pathomechanism of the disease, driven by a gain-of-function mutation in the NLRP3 gene. Familial Mediterranean fever (FMF) is a auto-inflammatory disorder characterised by recurring, self-limited episodes of fever and serositis resulting in abdominal, chest, joint and muscular pain due to mutation of MEFV. Colchicine is generally safe, nevertheless, 5-10% patients do not respond to treatment. IL-1blockade is the treatment of choice of CAPS and colchicine resistant FMF. IL-1 receptor antagonist-anakinra, human dimeric protein that incorporates the extra-cellular domain of IL-1 receptor and IL-receptor accessory protein-rilonacept, and a human anti-IL-1b monoclonal antibody-canakinumab are biologic agents used for this aim. We report the effect of anti-interleukin 1 treatment (canakinumab, anakinra) in 12 patients (2 CAPS cases and 10 FMF cases of resistant to colchicine therapy).