结缔组织病相关性肺动脉高压59例临床分析

摘要

Objective: To understand the incidence, clinical features and prognosis of connective tissue disease (CTD) associated pulmonary arterial hypertension (PAH) , increase awareness and attention about the disease. Methods: all cases with pulmonary arterial hypertension in 715 cases with connective tissue disease were analyzed retrospectively. Results;The overall incidence rate of CTD-associated PAH is about8. 3%. In 59 CTD-associated PAH cases, there are 47 female cases and 12 male cases, aged 23 to 95 years with the mean age of (57 ± 19) years. The duration is 0. 1 to 30 years, and the average duration is (7. 4 ±7. 3) years. A-mong these cases, Behcet s disease had the highest incidence of pulmonary hypertension; it is 19. 2% , followed by systemic lupus erythematosus (13. 7% ) , Sjogren's syndrome (13. 5% ) , rheumatoid arthritis (7. 4% ) , ar-teritis (3.5%, P < 0. 01) . Age and pulmonary artery pressure was negatively correlated (correlation coefficient r = - 0. 490, P < 0. 01) ; The proportions of interstitial lung disease, anti-nuclear antibody ( ANA) -positive rates, rheumatoid factor (RF) positive rates between PAH group and non-PAH group had statistical difference (P < 0. 05). Conclusion: PAH is a common complication in connective tissue disease. In our study, Behcet's disease and systemic lupus erythematosus had the highest incidence of PAH; earlier age of onset, more serious PAH; the patients with pulmonary fibrosis, elevated inflammatory indicators, positive ANA and RF are more likely to suffer CTD-associated PAH.u0000'%目的:了解结缔组织病(connective tissue diseases,CTD)相关的肺动脉高压(pulmonary arterial hypertension,PAH)的发生率、临床特点及预后,提高对该病的认识及重视.方法:从715例CTD患者中筛选出伴有PAH的患者59例,对其临床资料进行回顾性分析.结果:合并的PAH的总发生率约为8.3％.59例患者中女性47例,男性12例；年龄23～95岁,平均(57±19)岁；病程1个月～ 30年.其中白塞病合并PAH发生率最高,为19.2％,系统性红斑狼为13.7％、干燥综合征为13.5％、类风湿关节炎为7.4％、大动脉炎为3.5％(P＜0.01).发病年龄与肺动脉压力呈负相关(相关系数r=-0.490,P＜0.01),PAH组与非PAH组比较肺间质病变比例、抗核抗体(ANA)阳性率、类风湿因子(RF)阳性率共存差异有统计学意义(P＜0.05).结论:CTD中PAH是较为常见的并发症,本组研究提示,其中白塞病和系统性红斑狼疮发生率最高；发病年龄越早,PAH越严重；肺间质纤维化、炎性指标的升高、ANA及RF阳性提示CTD患者有更易发生PAH的可能.