幼年特发性关节炎关节外表现诊治进展

Progress in diagnosis and treatment of extra-articular manifestations of juvenile idiopathic arthritis

幼年特发性关节炎(JIA)是一组原因不明,以慢性关节炎为主要特征且临床异质性较强的儿童风湿性疾病。患儿病情多数预后较好,但部分患儿可有关节外重要组织器官受累,如心血管系统损害、肺胸膜病变、肾淀粉样变性和葡萄膜炎等。如果诊治延误可致近期和/或远期重要脏器功能损害,导致病情加重或遗留严重后遗症,引起生活质量下降甚至危及生命。因此,了解JIA关节外表现对疾病管理十分重要。

Characterized by chronic arthritis,juvenile idiopathic arthritis(JIA)refers to a group of rheumatic diseases in children with unknown causes and strong clinical heterogeneity.Most patients have good prognosis,while some may be involved with important extra-articular organs and tissues,including cardiac system damage,pulmonary or pleural lesions,renal amyloid degeneration,uveitis,and so on.Delay in diagnosis and treatment may lead to short-term and/or long-term involvement in vital systems,thus resulting in the aggravation of the disease or poor prognosis,which may decrease their life quality and even threaten their life.Understanding the extra-articular manifestations of JIA is very important to manage disease.