Koné-Paut Isabelle, Galeotti Caroline
a Division of Pediatric Rheumatology, Reference Center for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Le Kremlin Bicêtre Cedex, France.
Expert Rev Clin Immunol. 2015;11(10):1083-92. doi: 10.1586/1744666X.2015.1077702. Epub 2015 Aug 27.
Cryopyrin-associated periodic syndrome (CAPS) encompasses a spectrum of three phenotypes of increasing severity. The syndrome is due to dominant mutations in NLRP3, which encodes a key component of the innate immunity that regulates the secretion of IL-1β. CAPS manifests as systemic inflammation, which compromises quality of life and leads to serious complications and handicap. Anti-IL-1 drugs have shown remarkable efficacy in treating CAPS symptoms and have significantly changed patients' lives. They have acceptable safety profiles but do have some differences. We review three drugs that are currently marketed for CAPS, give additional information for the practical use of these drugs, and provide some recommendations for management.
冷吡啉相关周期性综合征（CAPS）包括三种严重程度递增的表型。该综合征是由NLRP3的显性突变引起的，NLRP3编码调节IL-1β分泌的先天免疫关键成分。CAPS表现为全身炎症，会损害生活质量并导致严重并发症和残疾。抗IL-1药物在治疗CAPS症状方面显示出显著疗效，并显著改变了患者的生活。它们具有可接受的安全性，但确实存在一些差异。我们综述了目前市场上用于治疗CAPS的三种药物，提供了这些药物实际应用的更多信息，并给出了一些管理建议。
