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Abstract：
BACKGROUND: Lung damage in systemic juvenile arthritis (sJIA) is one of the contemporary topics in pediatric rheumatology. Several previous studies showed the severe course and fatal outcomes in some patients. The information about interstitial lung disease (ILD) in the sJIA is scarce and limited to a total of 100 cases.
OBJECTIVE: To describe the features of sJIA patients with ILD in detail.
METHODS: In the present retrospective cohort study, information about 5 patients less than 18-years-old with sJIA and ILD were included. The diagnosis of sJIA was made according to the current 2004 and new provisional International League of Associations for Rheumatology criteria 2019. ILD was diagnosed with chest computed tomography with the exclusion of other possible reasons for concurrent lung involvement. Macrophage activation syndrome (MAS) was diagnosed with HLH-2004 and 2016 EULAR/ACR/PRINTO Classification Criteria and hScores were calculated during the lung involvement.
RESULTS: The onset age of sJIA ranged from 1 year to 10 years. The time interval before ILD ranged from 1 mo to 3 years. The disease course was characterized by the prevalence of the systemic features above articular involvement, intensive rash (100%), persistent and very active MAS (hScore range: 194-220) with transaminitis (100%), and respiratory symptoms (100%). Only 3 patients (60%) developed a clubbing phenomenon. All patients (100%) had pleural effusion and 4 patients (80%) had pericardial effusion at the disease onset. Two patients (40%) developed pulmonary arterial hypertension. Infusion-related reactions to tocilizumab were observed in 3 (60%) of the patients. One patient with trisomy 21 had a fatal disease course. Half of the remaining patients had sJIA remission and 2 patients had improvement. Lung disease improved in 3 patients (75%), but 1 of them had initial deterioration of lung involvement. One patient who has not achieved the sJIA remission had the progressed course of ILD. No cases of hyper-eosinophilia were noted. Four patients (80%) received canakinumab and one (20%) tocilizumab at the last follow-up visit.
CONCLUSIONS: ILD is a severe life-threatening complication of sJIA that may affect children of different ages with different time intervals since the disease onset. Extensive rash, serositis (especially pleuritis), full-blown MAS with transaminitis, lymphopenia, trisomy 21, eosinophilia, and biologic infusion reaction are the main predictors of ILD. The following studies are needed to find the predictors, pathogenesis, and treatment options, for preventing and treating the ILD in sJIA patients.
OBJECTIVE: To describe the features of sJIA patients with ILD in detail.
METHODS: In the present retrospective cohort study, information about 5 patients less than 18-years-old with sJIA and ILD were included. The diagnosis of sJIA was made according to the current 2004 and new provisional International League of Associations for Rheumatology criteria 2019. ILD was diagnosed with chest computed tomography with the exclusion of other possible reasons for concurrent lung involvement. Macrophage activation syndrome (MAS) was diagnosed with HLH-2004 and 2016 EULAR/ACR/PRINTO Classification Criteria and hScores were calculated during the lung involvement.
RESULTS: The onset age of sJIA ranged from 1 year to 10 years. The time interval before ILD ranged from 1 mo to 3 years. The disease course was characterized by the prevalence of the systemic features above articular involvement, intensive rash (100%), persistent and very active MAS (hScore range: 194-220) with transaminitis (100%), and respiratory symptoms (100%). Only 3 patients (60%) developed a clubbing phenomenon. All patients (100%) had pleural effusion and 4 patients (80%) had pericardial effusion at the disease onset. Two patients (40%) developed pulmonary arterial hypertension. Infusion-related reactions to tocilizumab were observed in 3 (60%) of the patients. One patient with trisomy 21 had a fatal disease course. Half of the remaining patients had sJIA remission and 2 patients had improvement. Lung disease improved in 3 patients (75%), but 1 of them had initial deterioration of lung involvement. One patient who has not achieved the sJIA remission had the progressed course of ILD. No cases of hyper-eosinophilia were noted. Four patients (80%) received canakinumab and one (20%) tocilizumab at the last follow-up visit.
CONCLUSIONS: ILD is a severe life-threatening complication of sJIA that may affect children of different ages with different time intervals since the disease onset. Extensive rash, serositis (especially pleuritis), full-blown MAS with transaminitis, lymphopenia, trisomy 21, eosinophilia, and biologic infusion reaction are the main predictors of ILD. The following studies are needed to find the predictors, pathogenesis, and treatment options, for preventing and treating the ILD in sJIA patients.
摘要：
背景：系统性幼年性关节炎（sJIA）的肺损伤是小儿风湿病的当代主题之一。先前的几项研究显示了一些患者的严重病程和致命结局。sJIA中有关间质性肺病（ILD）的信息很少，并且仅限于100例。
目的：详细描述sJIA合并ILD患者的特征。
方法：在本回顾性队列研究中，纳入了5例小于18岁的sJIA和ILD患者的信息.sJIA的诊断是根据当前的2004年和新的国际风湿病学协会临时联盟2019标准进行的。通过胸部计算机断层扫描诊断ILD，排除了并发肺部受累的其他可能原因。巨噬细胞活化综合征（MAS）诊断为HLH-2004和2016年EULAR/ACR/PRINTO分类标准，并在肺部受累期间计算其评分。
结果：sJIA的发病年龄为1岁至10岁。ILD之前的时间间隔为1个月至3年。病程的特点是关节受累以上的全身特征的患病率，剧烈皮疹（100%），持续性和非常活跃的MAS（hScore范围：194-220）与转氨酶（100％），和呼吸道症状（100％）。只有3名患者（60％）出现了棍棒现象。所有患者（100％）在疾病发作时出现胸腔积液，4例患者（80％）出现心包积液。两名患者（40％）发展为肺动脉高压。在3例(60%)患者中观察到对托珠单抗的输注相关反应。一名21三体病患者有致命的病程。其余一半患者sJIA缓解，2例患者改善。肺部疾病改善3例（75%），但其中1例最初肺部受累恶化。一名未达到sJIA缓解的患者的ILD病程进展。没有注意到嗜酸性粒细胞增多的病例。在最后一次随访时，四名患者（80％）接受了canakinumab和一名（20％）tocilizumab。
结论：ILD是严重的危及生命的sJIA并发症，可能会影响不同年龄的儿童，不同时间间隔的疾病发作。广泛的皮疹，浆膜炎（尤其是胸膜炎），全面的MAS与转氨酶，淋巴细胞减少,21三体，嗜酸性粒细胞增多，和生物输注反应是ILD的主要预测因子。需要进行以下研究来找到预测因子，发病机制，和治疗选择，用于预防和治疗sJIA患者的ILD。
目的：详细描述sJIA合并ILD患者的特征。
方法：在本回顾性队列研究中，纳入了5例小于18岁的sJIA和ILD患者的信息.sJIA的诊断是根据当前的2004年和新的国际风湿病学协会临时联盟2019标准进行的。通过胸部计算机断层扫描诊断ILD，排除了并发肺部受累的其他可能原因。巨噬细胞活化综合征（MAS）诊断为HLH-2004和2016年EULAR/ACR/PRINTO分类标准，并在肺部受累期间计算其评分。
结果：sJIA的发病年龄为1岁至10岁。ILD之前的时间间隔为1个月至3年。病程的特点是关节受累以上的全身特征的患病率，剧烈皮疹（100%），持续性和非常活跃的MAS（hScore范围：194-220）与转氨酶（100％），和呼吸道症状（100％）。只有3名患者（60％）出现了棍棒现象。所有患者（100％）在疾病发作时出现胸腔积液，4例患者（80％）出现心包积液。两名患者（40％）发展为肺动脉高压。在3例(60%)患者中观察到对托珠单抗的输注相关反应。一名21三体病患者有致命的病程。其余一半患者sJIA缓解，2例患者改善。肺部疾病改善3例（75%），但其中1例最初肺部受累恶化。一名未达到sJIA缓解的患者的ILD病程进展。没有注意到嗜酸性粒细胞增多的病例。在最后一次随访时，四名患者（80％）接受了canakinumab和一名（20％）tocilizumab。
结论：ILD是严重的危及生命的sJIA并发症，可能会影响不同年龄的儿童，不同时间间隔的疾病发作。广泛的皮疹，浆膜炎（尤其是胸膜炎），全面的MAS与转氨酶，淋巴细胞减少,21三体，嗜酸性粒细胞增多，和生物输注反应是ILD的主要预测因子。需要进行以下研究来找到预测因子，发病机制，和治疗选择，用于预防和治疗sJIA患者的ILD。
