风湿性疾病合并纵隔气肿94例临床特征及死亡危险因素分析

Clinical characteristics and risk factors for mortality analysis of 94 cases of rheumatic diseases with pneumomediastinum

目的分析风湿性疾病合并纵隔气肿的临床特点和相关风险因素。方法回顾性分析北京协和医院与山西白求恩医院1998年1月至2018年10月风湿性疾病合并纵隔气肿的住院患者临床资料,患者分为炎性肌病组(IIM)和非炎性肌病组(non-IIM),比较2组患者的临床特点、实验室检查和治疗的区别,应用Cox风险比例模型分析风湿性疾病合并纵隔气肿死亡的风险因素。结果共纳入94例,男性45例(占48%),平均年龄(45±14)岁,其中IIM组62例,non-IIM组(包括SLE、RA、SS等)32例。全部患者中69例(73%)发生纵隔气肿前有诱发因素,最常见为剧烈咳嗽和机械通气。IIM组与non-IIM组相比,指端血管炎(29%和6%,χ^(2)=6.540,P=0.008)、关节炎发生率(60%和28%,χ^(2)=8.409,P=0.004)、间质性肺疾病(ILD)发生率(98%和78%,χ^(2)=11.129,P=0.002)显著升高,环磷酰胺治疗率更高(82%和62%,χ^(2)=4.458,P=0.035),2组患者住院期间病死率(分别为50%与59%,χ^(2)=0.745,P=0.388)、6个月后病死率(分别为64%与72%,χ^(2)=0.517,P=0.472),差异均无统计学意义(P均>0.05)。合并肺部感染是死亡的危险因素[HR=3.131,95%CI(1.025,9.561),P=0.045],其中以细菌感染最为常见(65/75,87%)。结论纵隔气肿是风湿性疾病的高危并发症,伴发纵隔气肿的风湿性疾病患者多表现为多系统受累,积极应用免疫抑制剂治疗时应充分权衡感染对预后的不利影响。

Objective To analyze the clinical characteristics and risk factors of rheumatic diseases complicated with Pneumomediastinum(PnM).Methods A retrospective analysis of 94 inpatients with rheumatic diseases associated PnM from Peking Union Medical College Hospital and Shanxi Bethune Hospital between January 1998 and October 2018 was carried out.Patients were divided into idiopathic inflammatory myopathies(IIM)and the non-IIM group.Clinical features,laboratory examinations and treatment were compared between the two groups.Cox proportional hazard model was used to investigate the risk factors for prognosis.Results A total of 94 patients were included in the study,with an average age of(45±14)years.Forty-five(48%)of them were male.There were 62 patients in the IIM group and the other 32 were in the non-IIM group.Sixty-nine patients had predisposing factors before PnM.Severe cough and assisted mechanical ventilation were the most common causes.Compared with the non-IIM group,the incidence of digital vasculitis(29%vs 6%,χ^(2)=6.540,P=0.008),arthritis(60%vs 28%,χ^(2)=8.409,P=0.004),interstitial lung disease(ILD)(98%vs 78%,χ^(2)=11.129,P=0.002)were higher in the IIM group,treatment with cyclophosphamide was higher in the IIM group(χ^(2)=4.458,P=0.035).There was non-significant difference in mortality between the two groups during hospitalization(50%vs 59%)and 6 months after PnM(64%vs72%)(P>0.05).Pulmonary infection was the only risk factor for poor prognosis[HR=3.131,95%CI(1.025,9.561),P=0.045],in which bacteria infection was the most common cause(65/75,86.7%).Conclusion PnM is a severe complication of rheumatic diseases.To get a good prognosis,rheumatol-ogists should balance the benefit and risk of infection of immunosuppressive therapy.