系统性硬化病相关肺动脉高压的临床特点

The clinical characteristics of systemic sclerosis-related pulmonary arterial hypertension

目的 通过对比系统性硬化病（SSc）相关肺动脉高压（PAH）和特发性肺动脉高压（IPAH）的临床资料,进一步明确SSc相关PAH的临床特点.方法 入选经右心导管确诊并除外其他疾病的SSc相关PAH者和IPAH者,比较其临床资料、6分钟步行距离（6MWD）、肺功能及血流动力学参数.结果 SSc相关PAH者20例,女19例,男1例,年龄（43.1 ±12.2）岁;IPAH者18例,女16例,男2例,年龄（38.4±12.4）岁.两者平均肺动脉压、心指数和肺血管阻力（PVR）差异无统计学意义.与IPAH者比,SSc相关PAH者的用力肺活量（FVC）占预计值百分比[（77.1±13.2）％比（88.6±14.9）％,P=0.026]、肺一氧化碳弥散量（DLCO）占预计值百分比[（46.2±13.1）％比（66.6±13.3）％,P＜0.001]、[DLCO/肺泡通气量（VA）]占预计值百分比[（55.1±14.3）％比（75.1±11.5）％,P＜0.001]降低,6MWD缩短[（365.6 ±85.1）m比（454.3±136.8）m,P=0.034].多因素分析显示,PVR升高（OR=2.122,95％ CI1.093 ～4.119,P=0.026）、DLCO占预计值百分比降低（OR=0.916,95％ CI0.842～0.996,P=0.040）是6MWD ＜400 m的独立预测因素.结论 在血流动力学参数相同的情况下,SSc相关PAH者的限制性通气障碍和弥散障碍较IPAH者更为多见.

Objective To study the clinical,cardiopulmonary functional and hemodynamic profiles of systemic sclerosis patients with pulmonary hypertension （SSc-PAH） compared with those of idiopathic pulmonary hypertension （IPAH）.Methods Patients diagnosed with SSc-PAH or IPAH by right heart catheterization were consecutively enrolled into the study between 2011 and 2013 in Peking Union Medical College Hospital （PUMCH）.Cases with pulmonary hypertension related to other diseases were excluded.Demographic data,laboratory parameters,6 minutes walk distance （6MWD）,pulmonary function and hemodynamic variables at the time of diagnosis were collected and compared between the two groups.Results A total of 20 SSc-PAH patients including 19 females and one male with age of （43.1 ± 12.2） years,and 18IPAH patients including 16 females and 2 males with age of （38.4 ± 12.4） years were enrolled in this study.Subjects in both groups had similar mean pulmonary arterial pressure,cardiac index and pulmonary vascular resistance （PVR） when recruited.Compared with IPAH patients,SSc-PAH patients showed significantly decreased all parameters including forced vital capacity （FVC）％ [（77.1 ± 13.2）％ vs （88.6 ± 14.9）％,P =0.026],diffusing capacity of the lung for carbon monoxide （DLCO） ％ [（46.2 ±13.1） ％ vs （66.6± 13.3）％,P ＜0.001],DLCO/alveolar ventilation（VA） [（55.1 ± 14.3）％ vs （75.1 ± 11.5）％,P ＜0.001],and 6MWD [（365.6 ±85.1） m vs （454.3 ± 136.8） m,P =0.034].In subgroup analysis of SSc-PAH patients,elevated PVR （OR 2.122,95％ CI 1.093-4.119,P =0.026） and decreased DLCO％ （OR 0.916,95％ CI 0.842-0.996,P =0.040） were independently associated with reduced 6MWD.Conclusions Under the similar hemodynamic condition,SSc-PAH patients had more severe restrictive ventilation dysfunction and diffusion capacity dysfunction.Decreased 6MWD in SSc-PAHpatients was probably related to the impairment of pulmonary function.