【摘要】 目的探讨结缔组织病合并肺动脉高压的临床、诊治和预后相关因素。方法回顾性分析131例结缔组织病并发肺动脉高压患者的临床表现、超声心动图、实验室检查特点、主要治疗方法和转归以及预后的相关因素。结果 131例并发肺动脉高压的结缔组织病患者中,系统性红斑狼疮、类风湿关节炎、干燥综合征人数最多,其中男20例,女111例;平均年龄(51. 2±17. 7)岁。入院时常规行心脏超声检查,测定肺动脉压平均值为(66. 1±25. 4) mm Hg。所有患者主要合并症为间质性肺病(31例)、肺部感染(27例)、合并高尿酸血症者21例(> 420μmol/L),系统性红斑狼疮占13例。治疗上,分为针对原发病结缔组织病的治疗,以及根据肺动脉高压的严重程度对应降肺动脉高压的治疗,出院后,连续随访1年,复诊时,再次行超声心动图检测肺动脉高压平均值为(46. 2±18. 9) mm Hg。与治疗前相比,治疗后肺动脉高压值下降明显,差异有统计学意义(P <0. 05)。进一步分析发现,合并高尿酸血症的结缔组织病患者治疗效果更佳,肺动脉高压下降更为显著。结论肺动脉高压是结缔组织病常见并发症,经分析,在我科住院治疗的结缔组织病同时合并肺动脉高压的患者中,系统性红斑狼疮人数最多,血尿酸水平可能在结缔组织病合并肺动脉高压患者中起一定作用,其与治疗与预后的相关性有待进一步研究。更多还原

【Abstract】 Objective To investigate the related factors with clinical diagnosis,treatment and prognosis of connective tissue disease complicated by pulmonary hypertension. Methods The clinical manifestations,echocardiography and laboratory examination characteristics, main treatment methods, outcomes, and related factors of prognosis of 131 patients with connective tissue disease who were treated in our hospital from April 2009 to August 2017 were retrospectively analyzed.Results Among the 131 patients with connective tissue disease complicated by pulmonary hypertension,The patients with systemic lupus erythematosus,rheumatoid arthritis and Sjogren syndrome were the most. The main complications were interstitial lung disease( 31 cases),pulmonary infection( 27 cases) and hyperuricemia( > 420μmol/L,21 cases). systemic lupus erythematosus( 13 cases). After 1-year follow up,the mean value of pulmonary artery pressure measured by echocardiography was( 46. 2 ± 18. 9) mm Hg,which was significantly lower than that( 66. 1 ± 25. 4) mm Hg before treatment.Moreover the decrease degree of pulmonary hypertension in patients with connective tissue disease complicated by hyperuricemia was more significant. Conclusion Among the patients with connective tissue diseases complicated by pulmonary hypertension,the patients with systemic lupus erythematosus are the most,and the blood uric acid levels may play a certain role in pathogenesis of connective tissue disease complicated by pulmonary hypertension,and the correlation between the treatment and prognosis needs to be researched further.更多还原