全身型幼年特发性关节炎及合并巨噬细胞活化综合征诊疗专家共识(2022版)

Exerts consensus on diagnosis and treatment of systemic juvenile idiopathic arthritis combined with macrophage activation syndrome(2022 version)

全身型幼年特发性关节炎(systemic juvenile idiopathic arthritis,sJIA)是幼年特发性关节炎的一个亚型,是一种以高热和皮疹为主要特征,可伴全身多脏器功能损害的炎症性疾病。巨噬细胞活化综合征(macrophage activation syndrome,MAS)是最常继发于sJIA的严重风湿性疾病并发症,进展迅速,诊治不及时可危及生命。sJIA合并MAS的病因目前尚不明确,早期临床特征缺乏特异性,容易漏诊或误诊,延误治疗时机。虽然近年来对sJIA合并MAS的认识逐渐深入,但如何及时、合理诊治该病,仍是儿童风湿病医师面临的挑战。为进一步规范sJIA及其合并MAS的诊断、治疗、减停药方案,改善疾病总体预后,由中华医学会儿科学分会风湿病学组等4个儿童风湿病专业学术组织联合制定《全身型幼年特发性关节炎及合并巨噬细胞活化综合征诊疗专家共识(2022版)》,以更好指导该类疾病的临床诊治。

Systemic juvenile idiopathic arthritis(sJIA)is a sub-type of juvenile idiopathic arthritis.It is an inflammatory disease characterized by high fever,rash and/or systemic multiple organ dysfunction.Macrophage activation syndrome(MAS)is a complication of rheumatic diseases.It is almost secondary to sJIA in children.At present,the etiology of sJIA and MAS is not clear,the early clinical features are lack of specificity.So it′s easily to miss diagnosis,misdiagnosis,delay the opportunity of treatment,even lead to death.Although the understanding of sJIA and MAS has been gradually deepened in recent years,how to timely and reasonably diagnose and treat it is still a common problem encountered by the children′s rheumatology doctors.In order to further standardize the diagnosis,treatment,drug reduction and withdrawal strategies of sJIA and sJIA combined with MAS,and improve the overall prognosis of the disease,four professional academic organizations of children′s rheumatology,including the Subspecialty Group of Rheumatology,the Society of Pediatrics,Chinese Medical Association,jointly formulated the"Exerts consensus on diagnosis and treatment of systemic juvenile idiopathic arthritis combined with macrophage activation syndrome(2022 version)",to better guide clinical diagnosis and treatment.