Cryopyrin-associated periodic syndromes: background and therapeutics.

Abstract

Cryopyrin-associated periodic syndromes (CAPS) are caused by mutations of the gene encoding the NLR family protein NLRP3, which together with caspase-1 and adaptor proteins constitutes a protein complex termed the inflammasome. In innate immune reactions, a variety of stimuli activate the NLRP3 inflammasome, triggering caspase-1 to process proIL-1 and thus to produce mature IL-1. Excessive production of IL-1 by monocytes/macrophages is the central pathophysiology of CAPS, and daily injection of the IL-1 receptor antagonist anakinra rapidly ameliorates the inflammatory symptoms in most cases. Furthermore, double-blind, placebo-controlled clinical trials have recently confirmed the efficacy and safety of rilonacept, a fusion protein of the IL-1 receptor and IgG Fc, and canakinumab, a human anti-IL-1 monoclonal antibody, as novel long-lasting agents for treating CAPS.

• Authors: Tetsuo Kubota, Ryuji Koike
• Journal: Modern rheumatology (Mod Rheumatol) Vol. 20 Issue 3 Pg. 213-21 (Jun 2010) ISSN: 1439-7609 [Electronic] England
• PMID: 20140476 (Publication Type: Journal Article, Review)

Chemical References

• Antibodies, Monoclonal
• Antibodies, Monoclonal, Humanized
• Carrier Proteins
• Interleukin-1
• NLR Family, Pyrin Domain-Containing 3 Protein
• NLRP3 protein, human
• Recombinant Fusion Proteins
• canakinumab
• rilonacept

Topics

• Antibodies, Monoclonal (therapeutic use)
• Antibodies, Monoclonal, Humanized
• Carrier Proteins (genetics, immunology)
• Cryopyrin-Associated Periodic Syndromes (genetics, immunology, therapy)
• Humans
• Interleukin-1 (antagonists & inhibitors, genetics, immunology)
• Monocytes (immunology)
• NLR Family, Pyrin Domain-Containing 3 Protein
• Recombinant Fusion Proteins (therapeutic use)