Schneider R, Laxer R M
Department of Paediatrics, University of Toronto, Ontario, Canada.
Baillieres Clin Rheumatol. 1998 May;12(2):245-71. doi: 10.1016/s0950-3579(98)80018-6.
Systemic onset juvenile rheumatoid arthritis (SoJRA) accounts for 10-20% of all JRA, affecting males and females equally and occurring most frequently under the age of 5 years. It is characterized by arthritis, daily spiking fever, an evanescent rash, serositis and a variety of other extra-articular features. Exclusion of systemic infections, malignancies and connective tissue diseases is most important in establishing the diagnosis. The disease has a wide range of severity from a short monocyclic course to a prolonged chronic course with severe destructive arthritis in approximately one third of patients. Destructive arthritis, secondary amyloidosis and treatment complications including infections, osteoporosis, growth retardation and the macrophage activation syndrome account for the significant morbidity and mortality associated with the disease. Pharmacological management includes non-steroidal anti-inflammatory drugs, corticosteroids, methotrexate and an emerging role for cyclosporine A and cytotoxic drug therapy. Elucidation of the immunopathogenetic mechanisms may lead to new targeted therapy.
全身型幼年类风湿关节炎（SoJRA）占所有幼年类风湿关节炎的10%-20%，男女发病率相同，最常见于5岁以下儿童。其特征为关节炎、每日高热、一过性皮疹、浆膜炎及多种其他关节外表现。在确立诊断时，排除全身感染、恶性肿瘤及结缔组织病最为重要。该病严重程度差异很大，从短期单循环病程到约三分之一患者出现的伴有严重破坏性关节炎的长期慢性病程。破坏性关节炎、继发性淀粉样变性以及包括感染、骨质疏松、生长发育迟缓及巨噬细胞活化综合征在内的治疗并发症，是导致该病显著发病和死亡的原因。药物治疗包括非甾体抗炎药、皮质类固醇、甲氨蝶呤，环孢素A和细胞毒性药物治疗也逐渐发挥作用。阐明免疫发病机制可能会带来新的靶向治疗方法。
