PDF(Pubmed)
Abstract：
Background: Pulmonary involvement in systemic juvenile idiopathic arthritis (SJIA) is a rare but dangerous complication. The main risk factors are already known, such as macrophage activation syndrome, a refractory course of systemic juvenile arthritis, infusion reaction to interleukin 1 and/or interleukin 6 blockers, trisomy 21, and eosinophilia. However, information about respiratory system involvement (RSI) at the onset of SJIA is scarce. Our study aimed to evaluate the specific features of children with SJIA with RSI and their outcomes. Methods: In a single-center retrospective cohort study, we compared the information from the medical records of 200 children with SJIA according to ILAR criteria or SJIA-like disease (probable/possible SJIA) with and without signs of RSI (dyspnea, shortness of breath, pleurisy, acute respiratory distress syndrome, and interstitial lung disease (ILD)) at the disease onset and evaluated their outcomes (remission, development of chronic ILD, clubbing, and pulmonary arterial hypertension). Results: A quarter (25%) of the SJIA patients had signs of the RSI at onset and they more often had rash; hepato- and splenomegaly; heart (pericarditis, myocarditis), central nervous system, and kidney involvement; hemorrhagic syndrome; macrophage activation syndrome (MAS, 44.4% vs. 9.0%, p = 0.0000001); and, rarely, arthritis with fewer active joints, compared to patients without RSI. Five patients (10% from the group having RSI at the onset of SJIA and 2.5% from the whole SJIA cohort) developed fibrosing ILD. All of them had a severe relapsed/chronic course of MAS; 80% of them had a tocilizumab infusion reaction and further switched to canakinumab. Unfortunately, one patient with Down\'s syndrome had gone. Conclusion: Patients with any signs of RSI at the onset of the SJIA are required to be closely monitored due to the high risk of the following fibrosing ILD development. They required prompt control of MAS, monitoring eosinophilia, and routine checks of night oxygen saturation for the prevention/early detection of chronic ILD.
摘要：
背景：系统性幼年特发性关节炎（SJIA）的肺部受累是一种罕见但危险的并发症。主要的危险因素是已知的，如巨噬细胞活化综合征，系统性青少年关节炎的难治性病程，对白细胞介素1和/或白细胞介素6受体阻滞剂的输注反应，21三体和嗜酸性粒细胞增多。然而,SJIA发病时呼吸系统受累（RSI）的信息很少。我们的研究旨在评估SJIA伴RSI儿童的具体特征及其结果。方法：在单中心回顾性队列研究中，根据ILAR标准或SJIA样疾病（可能/可能的SJIA），我们比较了200名SJIA儿童的医疗记录中的信息，呼吸急促,胸膜炎,急性呼吸窘迫综合征,和间质性肺病（ILD））在疾病发作时评估其结果（缓解，慢性ILD的发展，俱乐部,和肺动脉高压）。结果：四分之一（25％）的SJIA患者在发病时有RSI的迹象，并且更经常出现皮疹；肝和脾肿大；心脏（心包炎，心肌炎），中枢神经系统,和肾脏受累；出血综合征；巨噬细胞活化综合征(MAS，44.4%vs.9.0%，p=0.0000001)；以及很少,关节活动较少的关节炎，与没有RSI的患者相比。五名患者(10%来自在SJIA发作时具有RSI的组,2.5%来自整个SJIA队列)发展成纤维化ILD。他们都有严重的MAS复发/慢性过程；其中80％的人有托珠单抗输注反应，并进一步改用canakinumab。不幸的是,一位患有唐氏综合症的病人走了。结论：由于以下纤维化ILD发展的高风险，在SJIA发作时有任何RSI迹象的患者需要密切监测。他们要求对MAS进行迅速控制，监测嗜酸性粒细胞增多,和夜间氧饱和度的常规检查，以预防/早期发现慢性ILD。
