儿童寡肌病性皮肌炎并发肺间质病变和纵隔气肿1例报告

One case of childhood hypomyopathic dermatomyositis complicated with interstitial lung disease and pneumomediastinum

目的探讨儿童寡肌病性皮肌炎并发肺间质病变、纵隔气肿的临床特点及诊断和治疗。方法回顾性分析1例寡肌病性皮肌炎并发肺间质病变、纵隔气肿患儿的临床资料,并复习相关文献。结果患儿女性,10岁11个月,因面部红斑1个月入院,有活动后气促的表现,肌力Ⅴ级,肌张力正常;有典型皮疹;肌酸激酶稍高;肌电图提示肌源性损害;膝关节磁共振提示双侧股骨后方软组织少许条状STIR序列高信号;高分辨CT(HRCT)示肺间质病变、纵隔气肿。临床诊断为寡肌病性皮肌炎并发肺间质病变、纵隔气肿。予糖皮质激素、环磷酰胺、环孢素、吡菲尼酮及丙种球蛋白冲击等综合治疗,患儿最终死亡。结论对于合并肌酸激酶正常的寡肌病性皮肌炎患儿,需警惕并发肺间质病变、纵隔气肿可能,后者病死率高,应早期完善肺部CT检查,及早治疗。

Objective To investigate the clinical characteristics, diagnosis, and treatment of childhood hypomyopathic dermatomyositis complicated with interstitial lung disease and pneumomediastinum. Methods One case of childhood hypomyopathic dermatomyositis complicated with interstitial lung disease and pneumomediastinum was retrospectively analyzed. The related literatures were reviewed. Results A 10 years and 11 months old female patient with facial enythema one month had shortness of breath after activities, muscle strength of Ⅴ level, normal muscular tension and typical skin rash. The creatine kinase level was slightly higher. The electromyography showed myogenic damage. The knee MRI showed that the soft tissue of the posterior bilateral femur had high signal of STIR sequence. The chest high resolution computed tomography（HRCT） showed interstitial lung disease and pneumomediastinum. Thus, hypomyopathic dermatomyositis complicated with interstitial lung disease and pneumomediastinum was clinically diagnosed. Finally, the patient died after a combination of anticoagulation and immunosuppressive therapies. Conclusions The diagnosis of interstitial lung disease and pneumomediastinum should be suspected in the patients of hypomyopathic dermatomyositis with normal creatine kinase. The mortality of this disease is high. Pulmonary CT examination should be implemented promptly.