de Toro Santos F J, Verea-Hernando H, Montero C, Blanco-Aparicio M, Torres Lanzas J, Pombo Felipe F
Department of Pneumology, Juan Canalejo Hospital, La Coruña, Spain.
Respiration. 1995;62(1):53-6. doi: 10.1159/000196390.
We describe a 41-year-old patient with adult-onset dermatomyositis who developed persistent pneumomediastinum and severe subcutaneous emphysema due to end-stage interstitial lung disease. The diagnosis of dermatomyositis was based on proximal muscle weakness, electromyographic findings of inflammatory myopathy, and positive findings on muscle biopsy. Low levels of creatine kinase elevation were found at the time of diagnosis (a form of dermatomyositis which has been associated with a poor prognosis). The patient had no signs of cutaneous vasculitis. Despite treatment with prednisone and azathioprine, she died of intercurrent gram-negative sepsis 15 months after the diagnosis of dermatomyositis.
我们描述了一名41岁成人起病的皮肌炎患者，该患者因终末期间质性肺疾病出现持续性纵隔气肿和严重皮下气肿。皮肌炎的诊断基于近端肌无力、炎性肌病的肌电图表现以及肌肉活检的阳性结果。诊断时发现肌酸激酶升高水平较低（这是一种与预后不良相关的皮肌炎形式）。患者无皮肤血管炎体征。尽管接受了泼尼松和硫唑嘌呤治疗，但她在皮肌炎诊断15个月后死于并发的革兰氏阴性菌败血症。
