NLRP3炎症小体与儿童自身炎症性疾病研究进展

儿童自身炎症性疾病（AID）是难治性疾病之一，发病机制尚未完全明确。近年来大量研究表明，NLRP3炎症小体失调在儿童AID的发生、发展中具有重要作用。NLRP3炎症小体是细胞内的一种多蛋白复合物，它能激活半胱氨酸天冬氨酸特异蛋白酶1（caspase-1），进一步促进炎症因子IL-1β和IL-18的成熟和分泌，从而促进细胞凋亡，调节固有免疫应答。IL-1受体拮抗剂（Anakinra）和IL-1β单克隆抗体（Canakinumab）治疗儿童AID取得了较好的疗效。本文就NLRP3炎症小体在该类疾病发病机制中的研究进展作一综述。
Abstract: Autoinflammatory diseases (AID) in childhood is one of refractory diseases, whose pathogenesis is not completely clear. In recent years, a large number of studies have shown that NLRP3 inflammasome plays an important role in the development of AIDs in children. Inflammasome is a cytosolic multiprotein complex that can activate cysteinyl aspartate-specific protease-1 (caspase-1), to further promote the maturation and secretion of proinflammatory cytokines IL-1β and IL-18 as well as pyroptosis and regulate innate immune response. IL-1 receptor antagonist (Anakinra) and IL-1β monoclonal antibody (Canakinumab) have good therapeutic effects in children with AIDs. This article reviews the research progress of NLRP3 inflammasome in the pathogenesis of autoinflammatory diseases. Key words: Multiprotein complexes Autoimmune diseases Interleukin-1 beta Chromosome disorders Review