本文選題：臨床無肌病皮肌炎 + 肺間質(zhì)病變　； 參考：《臨床兒科雜志》2017年11期

【摘要】：目的分析兒童起病的臨床無肌病皮肌炎(CADM)合并肺間質(zhì)病變(ILD)的臨床特點。方法回顧1例成功治療的CADM合并ILD患兒的臨床資料,并檢索中英文數(shù)據(jù)庫,分析兒童CADM合并ILD的臨床特點、治療及預后。結(jié)果女性患兒,13歲,Gottron's征陽性,臨床無乏力癥狀,肌酸激酶、肌電圖均正常,胸部高分辨CT提示肺間質(zhì)性病變。經(jīng)積極加用糖皮質(zhì)激素聯(lián)合環(huán)磷酰胺治療,有所好轉(zhuǎn)。檢索國內(nèi)外報道兒童CADM合并ILD 3例,加該患兒共4例,起病年齡10~16歲;首發(fā)癥狀分別為Gottern's征3例、發(fā)熱2例;4例患兒的抗核抗體及抗Jo-1抗體均為陰性,2例抗Ro-52抗體陽性。3例文獻報道的患兒在發(fā)現(xiàn)ILD時無呼吸系統(tǒng)臨床癥狀,半年內(nèi)迅速進展為呼吸衰竭死亡。結(jié)論兒童CADM合并ILD可呈現(xiàn)為快速進展型肺間質(zhì)性病變,臨床疑診為CADM的患兒應完善肺部影像學檢查,如存在ILD應予糖皮質(zhì)激素聯(lián)合免疫抑制劑積極治療,c改善預后。
[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.
【作者單位】： 中國醫(yī)學科學院北京協(xié)和醫(yī)學院北京協(xié)和醫(yī)院兒科;
【分類號】：R725.9

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