We read with great interest the recent article by Saper et al 1 describing high mortality of systemic juvenile idiopathic arthritis (sJIA) patients affected by parenchymal lung disease (LD). LD with sJIA has also been associated with macrophage activation syndrome (MAS).2 While both MAS and LD complicating sJIA are known risk factors for mortality, an effective therapeutic strategy has not been established.3 4 The present case report highlights an exacerbated LD complication in an sJIA patient treated successfully with additional plasma exchange (PE). A 5-year-old boy was diagnosed with sJIA when presenting with arthritis, prolonged fever and a skin rash. His white cell count (WCC, 8.5×109/L), C-reactive protein (CRP, 4.8 mg/dL), ferritin (467 ng/mL) and interleukin (IL)-18 (25 453 pg/mL) levels were elevated on diagnosis. Initial treatment of oral prednisolone at 18 mg/day and oral methotrexate at 6 mg/week was insufficient. Oral cyclosporine was started followed by tocilizumab, but …

中文翻译：

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血浆置换治疗难治性全身性幼年特发性关节炎合并巨噬细胞活化综合征和重症肺病的成功治疗

我们非常感兴趣地阅读了 Saper 等人最近发表的一篇文章 1 ，该文章描述了受肺实质疾病 (LD) 影响的全身性幼年特发性关节炎 (sJIA) 患者的高死亡率。伴有 sJIA 的 LD 也与巨噬细胞活化综合征 (MAS) 相关。2 虽然 MAS 和 LD 合并 sJIA 都是已知的死亡风险因素，但尚未建立有效的治疗策略。3 4 本病例报告强调了加重的 LD 并发症一名 sJIA 患者通过额外的血浆置换 (PE) 成功治疗。一名 5 岁男孩在出现关节炎、长时间发烧和皮疹时被诊断出患有 sJIA。他的白细胞计数 (WCC, 8.5×109/L)、C 反应蛋白 (CRP, 4.8 mg/dL)、铁蛋白 (467 ng/mL) 和白细胞介素 (IL)-18 (25 453 pg/mL) 水平分别为诊断时升高。口服泼尼松龙 18 毫克/天和口服甲氨蝶呤 6 毫克/周的初始治疗是不够的。开始口服环孢素，然后是托珠单抗，但是……