Familial cold-induced autoinflammatory syndrome, Muckle-Wells syndrome and neonatal-onset multisystem inflammatory disease make up cryopyrin-associated periodic syndromes (CPAS). These ore autoinflammatory inherited disorders caused by autosomal dominant gain-of-function mutations in the NLRP3 gene, located on chromosome 1q44. Cryopyrin/NALP3/NLRP3 is on essential component of intracellular inflammasomes that activate caspase-7, which in turn converts interleukin-10 (IL-10) to its active form. IL-10 is a potent cytokine that activates diverse elements of the immune and inflammatory systems leading to the pathogenic changes characteristic of CAPS. There is therefore much interest in the development of IL-10 blocking agents as novel biologic treatments for these conditions. Canakinumab (ACZ-885; llaris (R) Novortis Pharma) is a fully humanized monoclonal antibody (mAb) specific for IL-1 beta and is indicated for a wide range of inflammatory disorders including CAPS. This review will assess the utility of canakinumab as a treatment for CAPS.
- anti-IL-1-beta monoclonal-antibody
- inflammatory disorders