儿童临床无肌病皮肌炎合并肺间质病变1例报告及文献复习

发布时间：2018-05-10 14:17

本文选题：临床无肌病皮肌炎 + 肺间质病变　；参考：《临床儿科杂志》2017年11期

【摘要】：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,c改善预后。
[Abstract]:Objective to analyze the clinical features of pulmonary interstitial lesion (ILD) in children with non-myomyositis. Methods the clinical data of one child with CADM combined with ILD were reviewed, and the clinical features, treatment and prognosis of children with CADM combined with ILD were analyzed by searching Chinese and English databases. Results the Gottron's sign was positive in female children aged 13 years. There were no symptoms of fatigue, creatine kinase and electromyography were normal in the clinic. The lung interstitial lesions were revealed by chest high-resolution CT. After positive addition of glucocorticoid combined with cyclophosphamide treatment, some improvement. Three cases of CADM combined with ILD were reported at home and abroad, including 4 cases, the onset age of which was 1016 years old, the initial symptom was Gottern's sign in 3 cases, the first symptom was Gottern's sign in 3 cases, and the first symptom was Gottern's sign in 3 cases. In 2 cases of fever and 4 cases of fever, both antinuclear antibody and anti Jo-1 antibody were negative and 2 cases were positive for anti Ro-52 antibody. There were no clinical symptoms of respiratory system in the discovery of ILD, and rapid progression to respiratory failure death within half a year. Conclusion Children with CADM combined with ILD can present with rapid progressive pulmonary interstitial disease. Children suspected of CADM should improve their lung imaging. If there is ILD, glucocorticoid combined with immunosuppressant should be used to improve the prognosis.
【作者单位】：中国医学科学院北京协和医学院北京协和医院儿科;
【分类号】：R725.9

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