多发性肌炎/皮肌炎合并纵隔气肿的临床分析

Clinical analysis of pneumomediastinum complicated in polymyositis and dermatomyositis

目的研究多发性肌炎/皮肌炎(PM/DM)并发纵隔气肿的临床特点,探讨其发病危险因素及致病机制。方法回顾性分析了447例PM/DM患者的临床资料,包括年龄、性别、皮肤血管炎、肌酸激酶峰值、胸部影像学改变、纵隔气肿并发症、气管插管、治疗经过和预后。同时复习文献报道的17例DM合并纵隔气肿病例,进行了汇总分析。结果本组447例PM/DM患者中有4例(0·9%)并发纵隔气肿。本组和文献报道的共21例DM并发纵隔气肿的患者,年龄均偏小(平均34岁),男性较多(男∶女=13∶8),与DM未合并纵隔气肿患者比较差异均有统计学意义(P<0·05和P<0·01)。在21例患者中13例(61·9%)有明显的皮肤血管炎表现,而无纵隔气肿并发症的443例患者中只有44例(9·9%)有皮肤血管炎表现,两者比较差异有统计学意义(P<0·01)。肺间质病变在纵隔气肿患者中的发生率显著高于其他患者(P<0·01)。本组4例并发纵隔气肿的患者有3例接受气管插管治疗,而无纵隔气肿并发症的443例患者中只有5例(1·1%)接受气管插管,两者比较差异有统计学意义(P<0·01)。结论DM患者的血管病变可能是导致纵隔气肿发生的直接病因,肌炎症状较轻的年轻男性患者易发生纵隔气肿,肺间质病变和气管插管介入操作可能是纵隔气肿发生的危险因素。

Objective To analyze the clinical features of pneumomediastinum complicated in polymyositis and dermatomyositis （PM/DM） and to study the pathogenesis thereof. Methods The clinical data of 4 patients with pneumomediastinum complicated in dermatomyositis out of 447 PM/DM patients hospitalized in Peking Union Medical College （PUMC） Hospital Jan 1989 to June 2005, were analyzed. The records of patients with PM/DM available in English throughout the world were reviewed to collect those with pneumomediastinum as a complication. And the data of these patients were analyzed, focusing mainly on the age , gender, peak of serum creatine kinase （CK）, presence of pneumomediastinum, cutaneous vasculopathy, chest radiographic changes, tracheal cannula, management, and outcome. Results Among the 447 patients with PM/DM hospitalized in PUMC Hospital, 134 males and 313 females, aged 42 ± 17, pneumomediastinum was observed as a complication in four patients, 3 males and 1 female, aged 12-43, with a prevalence rate of 0.9%. Together with 17 cases reported in the English literatures there were 21 patients with pneumomediastinum complicated in polymyositis and dermatomyositis （PM/DM in all. Only one of the literatures reported a prevalence rate as high as 8.3% （4/48） , and other literatures were merely case reports. Compared with the PM/DM patients without pneumomediastinum the mean age of the PM/DM patients with pneumomediastinum was significantly younger （34： 42, P 〈 0.01 ）, the male： female ratio significantly higher （13：8 to 132：311, P 〈 0.01 ）, the morbidity rates of interstitial lung disease and of cutaneous vasculopathy significantly higher （ 18/21 to 134/443, and 12/21 to 44/443, both P 〈 0.01 ）. Although statistic analysis could not be undertaken because of the peak of CK not being provided in details in the literatures, the CK levels of the patients with pneumomediastinum were mostly normal or mildly higher with a peak lower than 500 U/L Three of the 4 patients with pneumomediastinum hospitalized in PUMC Hospital and 5 of the 443 patients （ 1.1% ） without this complication received tracheal cannula. There was a significant association of pneumomediastinum with tracheal cannula （P = 0. 000 ）. Conclusion Vasculopathy is strongly suspected as being responsible for the pneumomediastinum in DM, and male gender, younger age, interstitial lung disease, and tracheal cannu]a may be the risk factors of this pneumomediastinum complicated in PM/DM.