Autoinflammatory diseases (AIDs) are a group of disorders characterized by dysfunction of innate immunity which caused by gene mutations leading to coded proteins changes, finally causing uncontrolled systemic inflammation. AIDs are a group of rare rheumatic and inflammatory diseases. Here, Chinese Rheumatology Association summarized manifestations of the main AIDs, and to standardize the methods for diagnosis of AIDs.自身炎症性疾病(AIDs)是一组因基因突变致其编码蛋白改变、固有免疫失调,最终导致机体出现全身或器官炎症反应的疾病。AIDs是一组罕见的风湿性疾病,为提高临床医生对AIDs的认识与诊治水平,中华医学会风湿病学分会在借鉴目前国内外经验和指南的基础上,制定了本规范,总结了几种主要AIDs的临床表现,并规范了AIDs的诊断思路和方法。.