Abstract: Systemic juvenile idiopathic arthritis（sJIA）has a unique disease background and clinical profile，with various extra-articular manifestations and disease patterns. Its etiology remains unclear. The interaction between genetic and environmental factors contributes to the onset and progression of the disease. Inflammation control and targeted therapies are key to achieving disease remission. This review outlines the pathogenesis of sJIA，classification criteria，current research advances in diagnosis and treatment，and the main global principles for targeted therapeutic management，highlighting the importance of early diagnosis，precise intervention，and long-term follow-up in improving patient outcomes and quality of life，which helps to deepen clinicians’ overall understanding of sJIA.

Key words: systemic juvenile idiopathic arthritis, mechanism, classification diagnosis, targeted therapy

摘要： 全身型幼年特发性关节炎（systemic juvenile idiopathic arthritis，sJIA）具有独特的疾病背景和临床特点，有多种关节外表现和疾病模式。sJIA病因尚未明确，存在先天与后天因素的共同作用。炎症控制和靶向治疗是实现疾病缓解的关键。文章主要介绍了sJIA的发病机制、分类标准及目前诊疗相关研究进展，全球主要达标治疗管理原则，强调了早期诊断、精准干预及远期随访对改善患者预后、提高生活质量的重要作用，有助于进一步加深临床医生对sJIA的整体认识。

关键词: 全身型幼年特发性关节炎, 机制, 分类诊断, 靶向治疗