Tong Sheng-quan, Shi Xu-hua, Su Jin-mei, Zhao Yan, Zhang Feng-chun
Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Beijing 100730, China.
Zhonghua Yi Xue Za Zhi. 2006 Mar 7;86(9):624-7.
To analyze the clinical features of pneumomediastinum complicated in polymyositis and dermatomyositis (PM/DM) and to study the pathogenesis thereof.
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.
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).
Vasculopathy is strongly suspected as being responsible for the pneumomediastinum in DM, and male gender, younger age, interstitial lung disease, and tracheal cannula may be the risk factors of this pneumomediastinum complicated in PM/DM.
分析多发性肌炎和皮肌炎（PM/DM）合并纵隔气肿的临床特征并探讨其发病机制。
分析1989年1月至2005年6月在北京协和医院住院的447例PM/DM患者中4例合并纵隔气肿的皮肌炎患者的临床资料。检索全世界所有英文的PM/DM患者记录，收集合并纵隔气肿的病例，并对这些患者的数据进行分析，主要关注年龄、性别、血清肌酸激酶（CK）峰值、纵隔气肿的存在情况、皮肤血管病变、胸部X线改变、气管插管、治疗及预后。
在北京协和医院住院的447例PM/DM患者中，男性134例，女性313例，年龄42±17岁，4例患者出现纵隔气肿并发症，其中男性3例，女性1例，年龄12 - 43岁，患病率为0.9%。加上英文文献报道的17例，共有21例PM/DM合并纵隔气肿患者。仅有一篇文献报道患病率高达8.3%（4/48），其他文献均为病例报告。与无纵隔气肿的PM/DM患者相比，合并纵隔气肿的PM/DM患者平均年龄显著更小（34:42，P < 0.01），男女比例显著更高（13:8至132:311，P < 0.01），间质性肺病和皮肤血管病变的发病率显著更高（18/21至134/443，以及12/21至44/443，均P < 0.01）。尽管由于文献中未详细提供CK峰值而无法进行统计学分析，但纵隔气肿患者的CK水平大多正常或轻度升高，峰值低于500 U/L。在北京协和医院住院的4例纵隔气肿患者中有3例以及443例无此并发症患者中的5例（1.1%）接受了气管插管。纵隔气肿与气管插管之间存在显著关联（P = 0.000）。
强烈怀疑血管病变是DM中纵隔气肿的原因，男性、年轻、间质性肺病和气管插管可能是PM/DM合并纵隔气肿的危险因素。
