皮肌炎并发皮下、纵隔气肿6例临床分析并文献复习

目的：提高对皮肌炎（DM）并发皮下、纵隔气肿的各种临床相关因素的认识，重视纵隔气肿对血流动力学的影响。方法：将我院6例及国内文献报道7例DM并发纵隔气肿病例进行了汇总分析。结果：DM患者在并发纵隔气肿时，92％为自发性纵隔气肿。均有典型皮疹，61％有皮肤血管炎，54％肌力正常，69％肌酸激酶（CK）正常，92％乳酸脱氢酶（LDH）升高。上述病例均存在肺间质病变，且在激素治疗期间发生。38％出现循环系统的表现，53％死亡。结论：皮肌炎并发纵隔气肿以肺间质病变、皮肤血管炎病变为主而肌炎较轻、CK正常、LDH升高的皮肌炎患者更容易发生纵隔气肿，预后差，死亡率高。

Clinical analysis of dermatomyositis complicated by subcutaneous emphysema and pneumomediastinum and literature review

Objective： To get to know the clinical features of dermatomyositis （DM） complicated by subcutaneous emphysema and pneumomediastinum and the influence of pneumomediastinum on patients＇ hemodynamic status Methods： The clinical features of 6 hospitalized DM patients complicated by pneumomediastinum as well as 7 similar patients with clinical data in litera- tures were analyzed. Results： Typical rashes were found in all patients, with cutaneous vasculitis in 61% of the cases, normal muscle strength in 54% , normal CK in 69%, and increasing LDH in 92% . Interstitial lung changes were found before or while the complication was present, and steroids were in use in all the cases. Although no heart disease histories were recorded, cardiac vascular symptoms were found in 38% of the cases. The mortality rate was as high as 53%. Conclusion： DM patients with interstitial lung changes, cutaneous vasculitis, normal or mild myositis and CK and increased LDH were at the risk of pneumome-diastinum. The prognosis of such a complication was worse with high mortality rate.