Department of Pediatric Immunology Wilhelmina Children s Hospital, University Medical Center Utrecht, Lundlaan 6, 3584 EA, Utrecht, the Netherlands.
Best Pract Res Clin Rheumatol. 2009 Oct;23(5):655-64. doi: 10.1016/j.berh.2009.08.003.
Systemic juvenile idiopathic arthritis (sJIA) is a rare, systemic inflammatory disease classified as a subtype of JIA. Besides arthritis, it is characterised by systemic features such as spiking fever, skin rash, hepatosplenomegaly or serositis. It is becoming clear now that abnormalities in the innate immunity (cytokines such as interleukin (IL)-1, IL-6 and IL-18, and neutrophils and monocytes/macrophages rather than lymphocytes) play a major role in the pathogenesis of sJIA, distinguishing it from other JIA subtypes. Another distinctive feature of sJIA is its strong association with macrophage activation syndrome (MAS). Based on this, consensus is emerging that sJIA should be viewed as an autoinflammatory syndrome rather than a classic auto-immune disease. As a consequence of the progression in understanding the underlying mechanisms of sJIA, major changes in the management are evolving. So far, treatment has been based on glucocorticosteroids in combination with disease-modifying drugs such as methotrexate. Recently, remarkable improvement has been observed with IL-1 and IL-6 targeted therapies. These therapies might also change the long-term outcome of this disease. However, controlled trials set up in international collaboration are needed to determine the optimal treatment strategies for all sJIA patients.
全身型幼年特发性关节炎（sJIA）是一种罕见的全身性炎症性疾病，归类为幼年特发性关节炎的一个亚型。除关节炎外，其特征还包括全身症状，如高热、皮疹、肝脾肿大或浆膜炎。现在越来越清楚的是，先天免疫异常（细胞因子如白细胞介素（IL）-1、IL-6 和 IL-18，以及中性粒细胞和单核细胞/巨噬细胞，而不是淋巴细胞）在 sJIA 的发病机制中起主要作用，使其与其他幼年特发性关节炎亚型区分开来。sJIA 的另一个独特特征是其与巨噬细胞活化综合征（MAS）强烈相关。基于此，人们越来越认同 sJIA 应被视为自身炎症综合征，而不是经典的自身免疫性疾病。由于对 sJIA 潜在机制的理解不断深入，其治疗方法也在发生重大变化。迄今为止，治疗方法一直基于糖皮质激素联合甲氨蝶呤等疾病修饰药物。最近，针对白细胞介素 1 和白细胞介素 6 的靶向治疗取得了显著疗效。这些疗法也可能改变这种疾病的长期预后。然而，需要通过国际合作建立对照试验来确定所有 sJIA 患者的最佳治疗策略。
