目的 分析全身型幼年特发性关节炎合并肺部病变（sJIA-LD）患儿的临床特征、影像学表现、治疗及预后。方法 回顾性分析15例sJIA-LD患儿的临床资料,总结其临床特征、实验室指标、影像学改变及治疗预后。结果 15例患儿中男7例,女8例,平均发病年龄7岁。常见临床症状为发热15例,关节肿痛12例,咳嗽12例,皮疹11例,淋巴结肿大9例,浆膜炎8例,肝、脾肿大8例,气促7例,胸痛胸闷2例,腹痛1例。7例合并巨噬细胞活化综合征（MAS）。15例均行胸部HRCT检查,显示条索影或斑片影15例,实变影3例,网格影2例,结节影、钙化影、肺不张各1例;病变部位以左肺下叶最常见（13/15）。影像学检查结果符合机化性肺炎9例,非特异性间质性肺炎5例,寻常型间质性肺炎1例。13例应用非甾体类抗炎药及糖皮质激素。在诊断肺部病变时8例应用慢作用抗风湿药,其中环孢素3例、甲氨蝶呤4例、环磷酰胺1例,托珠单抗治疗8例。12例病情改善,复查胸部HRCT好转,1例肺部病变进展,最终死亡。结论 sJIA-LD早期临床症状轻微,死亡率高,胸部HRCT有助于早期诊断。

Objective To investigate the clinical features, imaging manifestations, treatment response and prognosis of children with systemic juvenile idiopathic arthritis associated lung disease (sJIA-LD). Methods The clinical data of 15 children with sJIA-LD were retrospectively analyzed, and the clinical characteristics, laboratory parameters, imaging changes and treatment prognosis were summarized. Results There were 7 boys and 8 girls in current study and the average age was 7 years. The common clinical symptoms were fever (15 cases), joint swelling and pain (12 cases), cough (12 cases), rash (11 cases), lymph node enlargement (9 cases), serositis (8 cases), hepatosplenomegaly (8 cases), shortness of breath (7 cases), chest pain (2 cases) and abdominal pain (1 case). Seven patients were complicated with macrophage activation syndrome (MAS). All the 15 children underwent chest high-resolution CT examination, and the results showed strip or patchy shadow in 15 children, consolidation shadow in 3 children, mesh shadow in 2 children, nodular shadow, calcification shadow and atelectasis in 1 child each. The commonest lesion site was the left lower lobe of the lung (13/15). The imaging findings revealed 9 cases of organizing pneumonia, 5 cases of non-specific interstitial pneumonia, and 1 case of usual interstitial pneumonia. All the 13 patients were treated with non-steroid anti-inflammatory drug and glucocorticoids. In the diagnosis of sJIA-LD, 8 cases were treated with slow-acting antirheumatic drugs, including 3 cases of cyclosporine, 4 cases of methotrexate and 1 case of cyclophosphamide. Eight children were treated with tocilizumab. The condition of 12 children improved, and chest high-resolution CT showed improvement. One patient had lung disease progression and eventually died. Conclusion The early clinical symptoms of sJIA-LD are mild but the mortality is high. The high-resolution CT is helpful for early diagnosis.