Woerner Andreas, von Scheven-Gête Annette, Cimaz Rolando, Hofer Michaël
Pediatric Rheumatology, University of Basel, University Children's Hospital, Basel, Switzerland.
Expert Rev Clin Immunol. 2015 May;11(5):575-88. doi: 10.1586/1744666X.2015.1032257. Epub 2015 Apr 4.
Systemic juvenile idiopathic arthritis (SJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. Systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that SJIA is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of SJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation, continue to be a major issue in SJIA patients' care. Translational research leading to a profound understanding of the cytokine crosstalk in SJIA and the identification of risk factors for SJIA complications will help to improve long-term outcome.
全身型幼年特发性关节炎（SJIA）是一种炎症性疾病，其特征为发热、淋巴结病、关节炎、皮疹和浆膜炎。全身炎症与先天性免疫系统失调有关，提示SJIA是一种自身炎症性疾病。白细胞介素-1（IL-1）和白细胞介素-6（IL-6）在SJIA的发病机制中起主要作用，使用IL-1和IL-6抑制剂进行治疗已显示出高效性。然而，SJIA的并发症，包括巨噬细胞活化综合征、关节炎导致的功能结局受限以及慢性炎症造成的长期损害，仍然是SJIA患者护理中的一个主要问题。开展转化研究以深入了解SJIA中的细胞因子相互作用并确定SJIA并发症的危险因素，将有助于改善长期结局。
