皮肌炎并发纵隔气肿的临床特点  

Clinical characteristics of dermatomyositis complicated with pneumomediastinum

作　　者：陈志涵[1] 高飞[1] 刘剑雯[1] 吴燕芳[1] 吴晨敏 林禾 CHEN Zhi-han;GAO Fei;LIU Jian-wen;WU Yan-fang;WU Chen-ming;LIN He(Department of Rheumatology,Fujian Provincial Hospital,Fujian Medical University,Fuzhou 350001,China)

机构地区：[1]福建医科大学福建省立医院风湿免疫科,福州350001

出　　处：《中华临床免疫和变态反应杂志》2018年第5期532-536,共5页Chinese Journal of Allergy & Clinical Immunology

摘　　要：目的分析皮肌炎(DM)并发纵隔气肿(PnM)的临床特点、治疗和转归。方法收集2012年至2017年在福建省立医院风湿免疫科治疗的60例DM患者的临床资料,详细分析5例DM并发PnM患者的临床特点,比较分析非PnM组与PnM组患者在年龄、性别、肌力、肌酸磷酸激酶(CK)峰值、肺部CT改变、治疗和预后方面的差异。结果 60例DM患者中5例并发PnM,占8. 33%,男2例,女3例;年龄42～56岁;包括经典皮肌炎2例和无肌病性皮肌炎(CADM) 3例,平均病程4. 6个月;治疗上都给予大剂量糖皮质激素联合免疫抑制剂;其中3例死亡,占60%,死亡时间是发生PnM后平均1. 8个月,2例死于严重肺部感染,1例死于呼吸衰竭。DM-PnM组患者与DM-非PnM组比较多为无肌力下降,差异有统计学意义(80. 0%比29. 1%,P=0. 038);且CK值较低,差异有统计学意义(57. 5 U/L比223 U/L,P=0. 023); DM-PnM组患者肺间质病变(100%比30. 9%)及肺部感染明显(80%比21. 8%),差异均有统计学意义(P=0. 005、P=0. 015)。DM-PnM组患者出现咽痛和声嘶临床表现者为80%,DM-非PnM组未出现相关症状。结论 DM并发PnM预后差,患者肌炎症状轻,易并发肺间质病变及肺部感染。DM患者出现咽痛和声嘶症状是发生PnM的高危因素。Objective To analyze the clinical characteristics,treatment,and prognosis of pneumomediastinum(PnM)complicated in dermatomyositis(DM).Methods The clinical data of 5 patients with PnM complicated in DM out of 60 patients were analyzed.The age,sex,muscle strength,serum creatine kinase(CK),radiology,treatment,and prognosis were compared between DM patients with or without PnM(PnM or non-PnM).Results The incidence of PnM in hospitalized DM patients was 8.33%.The ratio of male to female was∶3,while the age range was 42-56 years old.Two patients were classic DM and 3 were clinical amyopathic DM(CADM).The average disease duration was 4.6 months.All of the patients were given high-dose glucocorticoid and immunosuppressive therapy.Totally 3 patients(60%)died with an average of time of 1.8 months after the diagnosis.Two patients died of severe pulmonary infection,and 1 died of respiratory failure.When compared with non-PnM,PnM patients had no muscle weakness(80.0%vs.29.1%,P=0.038),a lower level of CK(57.5 U/L vs.223 U/L,P=0.023),and higher incidences of interstitial lung disease and pulmonary infection(100%vs.30.9%,P=0.005 and 80%vs.21.8%,P=0.015).Moreover,hoarseness and sore throat were much more frequently seen in PnM group(80%vs.0%,P<0.000).Conclusions DM patients complicated with PnM have a poor prognosis,and they usually have mild myositis,easily associated with interstitial lung disease and pulmonary infection.Hoarseness and sore throat may be the risk factors of DM patients complicated with PnM.

关 键 词：皮肌炎 纵隔气肿 临床特征 

分 类 号：R593.26[医药卫生—内科学] R564.3[医药卫生—临床医学]