结缔组织病并发肺动脉高压110例临床分析

Clinical analysis of 110 pulmonary arterial hypertension patients with connective tissue disease

目的：评估结缔组织病并发肺动脉高压的发生率、临床特点、诊断及治疗方法。方法：1278例结缔组织病患者中110例伴有肺动脉高压,对其进行回顾性分析。结果：结缔组织病合并肺动脉高压的发生率约为8.6%（110/1278）。110例患者中,女性95例,男性15例,年龄11～79岁,平均（46±17）岁;病程0.5～20年,平均（7±6）年。系统性硬化症（SSc）和混合性结缔组织病（MCTD）合并肺动脉高压发生率最高,分别为40.0%和25.0%,显著高于其他结缔组织病（P〈0.01）。有雷诺现象或雷诺现象合并肺间质病变者肺动脉压力显著升高（P〈0.01和P〈0.05）。且雷诺现象与肺动脉压力呈正相关（r=0.531,P〈0.01）。肺功能异常发生率较高,以弥散量降低最为多见。轻度肺动脉高压患者临床表现少,治疗可逆转;重度肺动脉高压治疗反应差,病死率高。结论：结缔组织病合并肺动脉高压较为常见,其中以SSc和MCTD合并肺动脉高压发生率最高。雷诺现象与肺动脉压力呈正相关,是预测肺动脉高压的良好指标。早期诊断和治疗,可以改善患者预后。

Objective To investigate the prevalence, clinical features, diagnosis and treatment of pulmonary arterial hypertension （PAH） in patients with connective tissue disease （CTD）. Methods All patients with pulmonary arterial hypertension in 1278 CTD patients were analyzed retrospectively. Results The prevalence of pulmonary arterial hypertension （PAH） in patients with connective tissue disease was about 8.6% （110/ 1278）. In these patients, 95 were female, 15 were male. The mean age was （46＋17） years, and the mean duration was （7＋6） years. Systemic sclerosis （SSc） and the mixed connective tissue disease （MCTD） had the highest incidence of PAH （40.0% and 25.0%） （P〈0.01）. Raynaud＇s phenomenon or Raynaud＇s phenomenon associated with interstitial lung disease were related to higher pulmonary arterial pressure （P〈0.01 and P〈0.05）. There was positive correlation （P〈0.01） between the presence of Raynaud＇s phenomenon and pulmonary arterial pressure. Abnormal lung function was a common finding. Patients with mild PAH had few clinical manifestations and good therapeutic responses, but severe PAH correlated with poor prognosis. Conclusion PAH is common in connective tissue disease. SSc and MCTD have the highest prevalence of PAH. The presence of Raynaud＇s phenomenon is positively correlated with pulmonary arterial pressure. It can predict the development of PAH.