Abstract: Systemic juvenile idiopathic arthritis （sJIA） is a subtype of juvenile idiopathic arthritis， which has unique clinical and pathological characteristics. Macrophage activation syndrome （MAS） most frequently occurs in sJIA. It progresses rapidly and can be life-threatening. The etiology of sJIA and sJIA with MAS remains unclear，which is a major challenge in childhood rheumatism. This review summarizes the progress in the diagnosis and treatment of sJIA， emphasizing the early warning，diagnosis and precise treatment， to reduce the rate of disability and mortality and improve the prognosis of the disease.

Key words: systemic juvenile idiopathic arthritis, macrophage activation syndrome, diagnosis, treatment

摘要： 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种亚型，具有独特的临床及病理特点。巨噬细胞活化综合征（macrophage activation syndrome，MAS）最常并发于sJIA，病情进展迅速，可危及生命。sJIA合并MAS的病因尚不明确，仍是目前儿童风湿病面临的巨大挑战。文章概述了sJIA诊治进展，强调疾病的早期预警、诊断及精准治疗，以降低致残致死率，改善疾病预后。

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, 治疗