儿童寡肌病性皮肌炎合并肺间质病变2例报告

发布时间：2018-04-24 06:37

本文选题：皮肌炎 + 寡肌病性　；参考：《临床儿科杂志》2017年06期

【摘要】：目的探讨托珠单抗对儿童寡肌病性皮肌炎合并肺间质病变的疗效。方法回顾分析2例寡肌病性皮肌炎合并肺间质病变患儿的临床特点及治疗与预后,并复习相关文献。结果男女各1例,女性患儿10岁11个月、男性患儿8岁5个月,起病时均有气促,但无肌肉损害的临床表现;均有典型皮疹,但肌力及肌张力正常。实验室检查以血清铁蛋白、乳酸脱氢酶、谷氨酸氨基转移酶及天冬氨酸氨基转移酶升高为主,肌酸肌酶除首次入院时稍高,复查始终在正常范围。高分辨CT示肺间质病变。临床诊断为寡肌病性皮肌炎合并肺间质病变。女性患儿经大剂量激素、环磷酰胺、环孢素、吡菲尼酮及丙种球蛋白等治疗无效死亡。男性患儿在常规激素治疗的基础上,加用托珠单抗(240 mg/次,2次),病情稳定,随访复查各指标均在正常范围内。结论儿童寡肌病性皮肌炎临床表现以及实验室检查结果不典型,死亡发生率高。联合托珠单抗治疗有效。
[Abstract]:Objective to investigate the effect of tozhu McAb on children with olivary dermatomyositis complicated with pulmonary interstitial disease. Methods the clinical features, treatment and prognosis of 2 cases of olivary dermatomyositis complicated with pulmonary interstitial disease were retrospectively analyzed. Results there were 1 case of male and female, 10 years and 11 months of female, 8 years and 5 months of male. All of them had shortness of breath but no muscle damage, and they all had typical rash, but the muscle strength and muscle tension were normal. Serum ferritin, lactate dehydrogenase, glutamate aminotransferase and aspartate aminotransferase were mainly increased in laboratory examination. High-resolution CT showed pulmonary interstitial lesions. Oligomyosis dermatomyositis with pulmonary interstitial disease was clinically diagnosed. Female children died after high dose of hormone, cyclophosphamide, cyclosporine, pifenidone and gamma globulin. On the basis of routine hormone therapy, male children were treated with tricuspid antibody 240 mg/ twice, the condition was stable, and every index of follow-up was within normal range. Conclusion the clinical manifestations and laboratory findings of children with oligomyopathy dermatomyositis are atypical and the incidence of death is high. Combined with tozumab is effective.
【作者单位】：苏州大学附属儿童医院;
【基金】：国家自然科学基金资助项目(No.81370787) 江苏省临床医学科技专项——新型临床诊疗技术攻关(No.SBL 2014030237)
【分类号】：R725.9

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