摘要
自身炎症性疾病(autoinflammatory diseases, AIDs)是由基因突变引起其编码蛋白发生改变, 造成固有免疫系统失调而引起全身炎症反应的一组疾病。这组疾病以反复或持续的炎症反应为特点(急性时相炎症蛋白升高)、缺乏适应性免疫系统的参与(缺乏自身反应性T淋巴细胞和自身抗体)。AIDs种类繁多, 临床表现和发病机制多样, 根据其临床特点及发病机制分为不同种类。对于起病年龄小、具有反复发作性的发热和其他全身炎症反应表现及家族史的患儿需考虑到AIDs的可能, 明确诊断依赖于病史、临床表现和基因检测结果的综合分析。治疗AIDs的常用药物包括非甾体类抗炎药物、糖皮质激素、免疫抑制剂和生物制剂。早期诊断并积极治疗AIDs可有效减轻全身炎症、缓解脏器损伤、降低远期并发症的发生率。为规范AIDs的诊治, 中华医学会儿科学分会风湿病学组等特制定儿童AIDs诊断与治疗专家共识。
Autoinflammatory diseases(AIDs)is a group of diseases caused by gene mutation,which changes its coding protein,causes the imbalance of innate immune system and leads to systemic inflammatory response.This group of diseases is characterized by repeated or continuous inflammatory response(increased acute phase inflammatory protein)and lack of participation of the adaptive immune system(lack of autoreactive T cells and autoantibodies).AIDs have a wide variety of clinical manifestations and pathogenesis.They can be divided into different types according to clinical characteristics and pathogenesis.For patients with young onset age,recurrent fever,other systemic inflammatory manifestations and family history,the possibility of AIDs should be considered.It is clear that the diagnosis depends on the comprehensive analysis of medical history,clinical manifestations and gene test results.Drugs for the treatment of AIDs include non steroidal anti-inflammatory drugs,glucocorticoids,immunosuppressants and biological agents.Early diagnosis and active treatment of AIDs can effectively reduce systemic inflammation,alleviate organ injury and reduce the incidence of long-term complications.In order to standardize the diagnosis and treatment of AIDs,the Subspecialty Group of Rheumatology,the Society of Pediatrics,Chinese Medical Association specially formulated the experts consensus on the diagnosis and treatment of AIDs in children.
作者
中华医学会儿科学分会风湿病学组
中国医师协会风湿免疫科医师分会儿科学组
海峡两岸医药卫生交流协会风湿免疫病学专业委员会儿童学组
福棠儿童医学发展研究中心风湿免疫专业委员会
李彩凤
张俊梅
邓江红
檀晓华
The Subspecialty Group of Rheumatology,the Society of Pediatrics,Chinese Medical Association;The Pediatric Group of Chinese Medical Doctor Association Committee on Rheumatology and Immunology;The Pediatric Group of Rheumatology and Immunology Professional Committee of Cross Strait Medical and Health Exchange Association;Rheumatology and Immunology Professional Committee of Futang Children′s Medical Development Research Center;Li Caifeng
出处
《中华实用儿科临床杂志》
CAS
CSCD
北大核心
2022年第3期161-172,共12页
Chinese Journal of Applied Clinical Pediatrics
基金
北京市医院管理中心儿科学科协同发展中心专项经费资助(XTCX201819)。
关键词
自身炎症性疾病
诊断
治疗
共识
Autoinflammatory disease
Diagnosis
Treatment
Consensus
