结缔组织病相关肺动脉高压临床分析263例  

Clinical analysis of 263 patients with connective tissue disease-associated pulmonary arterial hypertension

作　　者：姜波[1] 金艳盛[2] 王春[1] 周康兴[1] 冯学兵[1] Jiang Bo;Jin Yansheng;Wang Chun;Zhou Kangxing;Feng Xuebing(Department of Rheumatology and Immunology,Gulou Hospital Affiliated to Medical College of Nanjing University,Nanjing 210008,China;Department of Rheumatology and Immunology,Wuzhong People's Hospital,Suzhou 215128,China)

机构地区：[1]南京大学医学院附属鼓楼医院风湿免疫科,南京210008 [2]苏州市吴中人民医院风湿免疫科,江苏苏州215128

出　　处：《中华临床医师杂志（电子版）》2020年第10期764-768,共5页Chinese Journal of Clinicians(Electronic Edition)

基　　金：中华医学会临床医学科研专项资金(Z-2014-06-2-1863);南京市科技发展计划项目(201715021)。

摘　　要：目的总结结缔组织病相关肺动脉高压(CTD-PAH)患者的临床特征,探索CTDPAH发病的高危因素。方法收集2003年1月至2017年12月在南京鼓楼医院风湿免疫科住院的经胸心脏超声发现肺动脉压>35 mmHg(1 mmHg=0.133 kPa)的CTD患者263例,回顾性分析患者的临床资料,包括流行病学、症状体征、用药和检测指标。结果CTD-PAH患者年龄集中于19~65岁(77.19%,203/263),女性为主(83.7%,220/263);所有CTD-PAH患者中,系统性红斑狼疮(SLE)比例最高(38%),干燥综合征(29%)和系统性硬化症(11%)次之;CTD-PAH患者最常见的PAH表现为胸闷(77.57%)、气喘(71.86%)、咳嗽(61.98%),原发病表现以雷诺现象(56.27%)、口干(47.53%)、面部红斑等皮疹(42.97%)等最多见;CTD-PAH最常见合并症为间质性肺炎(41.44%)、心功能不全(32.32%)及蛋白尿(25.48%);CTD-PAH患者免疫抑制剂中羟氯喹(62.36%)和环磷酰胺(48.67%)使用率最高。结论CTD-PAH多发于中年女性,SLE及干燥综合征最多见,雷诺现象、口干、皮疹及间质性肺炎为其高危因素。Objective To explore the high risk factors for connective tissue diseases-associated pulmonary hypertension(CTD-PAH)by summarizing the clinical characteristics of CTD-PAH patients.Methods A total of 263 hospitalized patients with CTD whose pulmonary artery pressure was>35 mmHg(1 mmHg=0.133 kPa),as measured by transthoracic echocardiography were enrolled at the Department of Rheumatology and Immunology,Nanjing Gulou Hospital from January 2003 to December 2017.Their clinical data including epidemiology,symptoms and signs,medications,and laboratory indexes were studied retrospectively.Results Most CTD-PAH patients(77.19%,203/263)were diagnosed at 19-65 years old,and most were female(83.7%,220/263).Among all CTD-PAH cases,the proportion of systemic lupus erythematosus(SLE)was the highest(38%),followed by Sjogren's syndrome(29%)and systemic sclerosis(11%).The most common clinical manifestations related with PAH in CTD-PAH patients were chest tightness(77.57%),asthma(71.86%),and cough(61.98%).The most popular manifestations of primary CTD were Reynolds phenomenon(56.27%),dry mouth(47.53%),and rash(42.97%).The most common complications of CTD-PAH were interstitial lung disease(41.44%),cardiac insufficiency(32.32%),and proteinuria(25.48%).Hydroxychloroquine(62.36%)and cyclophosphamide(48.67%)were the most frequently used medications among immunosuppressants for CTD-PAH patients.Conclusion CTD-PAH predominantly occurs in middle-aged women,and SLE and Sjogren's syndrome are the most common.Reynolds phenomenon,dry mouth,rash,and interstitial lung disease are high risk factors for CTD-PAH.

关 键 词：肺动脉高压 结缔组织病 流行病学 临床特征 

分 类 号：R544.1[医药卫生—心血管疾病]