目的　分析儿童起病的临床无肌病皮肌炎（CADM）合并肺间质病变（ILD）的临床特点。方法　回顾1例 成功治疗的CADM合并ILD患儿的临床资料，并检索中英文数据库，分析儿童CADM合并ILD的临床特点、治疗及预后。 结果　女性患儿，13岁，Gottron's征阳性，临床无乏力症状，肌酸激酶、肌电图均正常，胸部高分辨CT提示肺间质性病变。 经积极加用糖皮质激素联合环磷酰胺治疗，有所好转。检索国内外报道儿童CADM合并ILD 3例，加该患儿共4例，起病 年龄10~16岁；首发症状分别为Gottern's征3例、发热2例； 4例患儿的抗核抗体及抗Jo-1抗体均为阴性， 2例抗Ro-52抗 体阳性。 3例文献报道的患儿在发现ILD时无呼吸系统临床症状，半年内迅速进展为呼吸衰竭死亡。结论　儿童CADM合 并ILD可呈现为快速进展型肺间质性病变，临床疑诊为CADM的患儿应完善肺部影像学检查，如存在ILD应予糖皮质激 素联合免疫抑制剂积极治疗，改善预后。

Objective　To analyze the clinical features of juvenile-onset clinically amyopathic dermatomyositis complicated by progressive interstitial pneumonia. Methods　A retrospective analysis of a case of juvenile-onset clinically amyopathic dermatomyositis on clinical features, diagnosis and treatment was performed. Data of the other three reported cases were also reviewed. Results　The patient was an adolescent girl presented with Gorrton's sign. The patient did not have fatigue and got normal result in creatine kinase and elctromyogram test. The HRCT exam showed interstitial pneumonia. The mean age of the four cases at the time of onset is 12.3 years old. Gottron's sign (3/4) and fever (2/4) are the most common symptoms of onset. Anti-nuclear antibody (ANA), anti-Jo-1 antibody are 100% negative in the four patients. Two of the four patients who received anti-Ro-52 antibody test are both positive. Three of the four patients were asymptomatic when the CT scan showed interstitial pneumonia. The interstitial pneumonia was progressive and three of the four patients died of respiratory failure within six months. Treatment with glucocorticoid and immunosuppressant was successful in one case. Conclusions　Juvenile CADM can be complicated by progressive interstitial pneumonia. Children suspected CADM should perform pulmonary imaging examinations to find interstitial pneumonia. Children diagnosed as CADM complicated by interstitial pneumonia should receive glucocorticoid and immunosuppressant treatment to prevent progression.