中国实用儿科杂志

中国实用儿科杂志 ›› 2024, Vol. 39 ›› Issue (6): 439-444.DOI: 10.19538/j.ek2024060607

• 专题笔谈 • 上一篇    下一篇

非炎症小体病诊治进展

  

  1. 深圳市儿童医院风湿免疫科，广东  深圳  518038
  • 出版日期:2024-06-06 发布日期:2024-07-11
  • 通讯作者: 杨军，电子信箱：rogasansz@163.com
  • 基金资助:
    国家重点研发计划（2021YFC2702001）；广东省高水平临床重点专科项目（SZGSP012）

Progress in diagnosis and treatment of non-inflammasom-apathies

  1. Department of Rheumatology and Immunology，Shenzhen Children's Hospital，Shenzhen  518038，China
  • Online:2024-06-06 Published:2024-07-11

摘要： 非炎症小体病是自身炎症性疾病中的一大类疾病，其囊括病种数较多，临床表现复杂多样。多数临床表现为反复发热、皮疹、关节炎症、眼部炎症，急性期炎症指标升高，根据临床线索需进一步完善基因检查明确诊断。目前尚无明确的非炎症小体病治疗指南，激素、非甾体类抗炎药、多种免疫抑制药物及生物制剂均有应用，但疗效各异。文章旨在介绍常见非炎症小体病发病机制、临床表现及诊治经验，以期提高临床医生对该类疾病认知。

关键词: 自身炎症性疾病, 非炎症小体病, 发热

Abstract: Non-inflammasomapathies is a group of autoinf-lammatory diseases. The clinical manifestations are complex. The common clinical manifestations are repeated fever， rash， arthritis， eye inflammation， and increased inflammatory protein in the acute phase. Genetic examination should be further improved to confirm the diagnosis. There are no guidelines for the treatment of non-inflammasomapathies. Glucocorticoids， non-steroidal anti-inflammatory drugs， immunosuppressives and biological agents have been applied， but the efficacy is different. In this review， the pathogenesis， clinical manifestations， diagnosis and treatment experience were discussed， so as to provide new insights for the understanding of these diseases. 

Key words: autoinflammatory diseases, non-inflammasoma-pathies, fever