全身型幼年特发性关节炎发生机制及诊治策略
Pathogenesis, diagnosis and treatment of systemic juvenile idiopathic arthritis
吴小川 1李灿琳1
作者信息
- 1. 410011长沙,中南大学湘雅二医院儿童医学中心
- 折叠
摘要
全身型幼年特发性关节炎(sJIA)典型临床表现为发热、皮疹、关节痛、多浆膜炎、肝和/或脾大,系幼年特发性关节炎(JIA)的一个亚型,发病率为6.6/100 000～ 15.0/100 000,但致残率和病死率占JIA的2/3以上.其症状和体征往往不十分典型,因此诊断常较困难.sJIA病因尚不明确,目前认为是一种自身炎症反应综合征,而非经典自身免疫性关节炎.但是经典自身炎症性疾病有明确致病基因及家族史,而sJIA的致病基因目前尚无定论,也无明显家族遗传性,这是二者最大区别.传统治疗包括非甾体类抗炎药、糖皮质激素和改变病情抗风湿药.但病情仍然凶险,易反复,并且易出现致命的并发症,即巨噬细胞活化综合征.现针对sJIA的病因及发病机制、临床表现、诊断、新型生物标志物、鉴别诊断及治疗进展进行阐述.
Abstract
The typical clinical manifestations of systemic juvenile idiopathic arthritis (sJIA) are fever,rash,arthralgia,polyserositis,hepatomegaly and/or splenomegaly.It is a subtype of JIA,the morbidity is 6.6/100 000-15.0/100 000,but the disability and mortality account for more than 2/3 of JIA.Its symptoms and signs are often not quite typical,therefore,it is often difficult to diagnose.The pathogenesis of sJIA is unclear at present,it is considered to be a self inflammatory response syndrome,rather than classical autoimmune arthritis.But,self inflammatory disease has a clear pathogenic gene and family history,the pathogenic gene of sJIA is still inconclusive,it also doesn't have obvious familial inheritance,this is the greatest difference.The traditional treatment for sJIA includes non steroidal anti-inflammatory drugs,glucocorticoids and disease modifying antirheumatic drugs.But,it is still dangerous and easy to relapse,what's more,it is prone to result in fatal complications-macrophage activation syndrome.This article will review the etiology,pathogenesis,clinical manifestations,diagnosis,new biomarkers,differential diagnosis and treatment of sJIA.
关键词
全身型幼年特发性关节炎/巨噬细胞活化综合征/诊断/新型生物标志物/治疗Key words
Systemic juvenile idiopathic arthritis/Macrophage activation syndrome/Diagnosis/New biomarkers/Treatment引用本文复制引用
出版年
2017
NETL
NSTL
维普
万方数据