Pathogenesis, diagnosis and treatment of systemic juvenile idiopathic arthritis
10.3760/cma.j.issn.2095-428X.2017.21.003
- VernacularTitle:全身型幼年特发性关节炎发生机制及诊治策略
- Author:
Xiaochuan WU
1
;
Canlin LI
Author Information
1. 中南大学湘雅二医院儿童医学中心
- Keywords:
Systemic juvenile idiopathic arthritis;
Macrophage activation syndrome;
Diagnosis;
New biomarkers;
Treatment
- From:
Chinese Journal of Applied Clinical Pediatrics
2017;32(21):1607-1612
- CountryChina
- Language:Chinese
-
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.