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自混合性结缔组织病的概念被提出以来，其发病机制及诊断一直备受争议。目前学术界普遍认为混合性结缔组织病是一种独立的风湿免疫病，其特征是系统性红斑狼疮、系统性硬化、皮肌炎/多肌炎一种或多种临床症状的混合，伴抗U1核糖核蛋白抗体高滴度阳性。有研究表明多达1/4的患者幼年起病，首发症状以雷诺现象和多关节炎为主，其临床特征和诊治特点与成人存在一定差异。疾病后期有进展为其他结缔组织病的可能，早期诊断和治疗对改善预后具有重要意义。该文就儿童混合性结缔组织病的流行病学、发病机制、临床表现及诊断治疗等方面的研究进展展开阐述。

Since the concept of mixed connective tissue disease was proposed，it remains controversial in its pathogenesis and diagnosis.Mixed connective tissue disease is generally recognized as a disease entity characterized by overlapping clinical features of systemic lupus erythematosus，systemic sclerosis，and polymyositis，as well as high titers of serum anti-U1 ribonucleoprotein antibody.Studies have shown that up to 1/4 of the patients have the disease at an early age，with the first symptoms of Raynaud's phenomenon and polyarthritis，and the disease has certain differences in clinical characteristics and diagnosis and treatment from adults.The disease may evolve into other connective tissue diseases in the later stage，so early diagnosis and treatment are of great significance to improve prognosis.This article elaborates on the epidemiology，pathogenesis，clinical manifestations，diagnosis and treatment of juvenile mixed connective tissue disease.