结缔组织病继发肺动脉高压的临床分析  

Clinical analysis of pulmonary arterial hypertension secondary to connective tissue disease

作　　者：姜莉[1] 薛愉[1] 程青青[1] 万伟国[1] 

机构地区：[1]复旦大学附属华山医院风湿免疫科,上海200040

出　　处：《临床内科杂志》2012年第8期540-542,共3页Journal of Clinical Internal Medicine

摘　　要：目的总结结缔组织病（CTD）继发肺动脉高压（PAH）患者的临床特点。方法回顾性分析1997—2011年复旦大学附属华山医院收治的53例结缔组织病继发肺动脉高压患者的临床资料，对患者的疾病种类、临床表现、实验室检查以及诊治和预后情况进行统计学分析。结果1530例结缔组织病患者共发生肺动脉高压53例，其中女46例，男7例，平均年龄（43．9±13．8）岁，病程（5．1±4．2）年。肺动脉高压在系统性红斑狼疮中最为常见。主要临床表现为呼吸困难（84．9％）和肢端雷诺征（56．6％）。肺动脉高压患者体内的ANA、nRNP和SSA抗体的比例明显升高。53例患者中7例（13．2％）死亡，死亡主要原因为右心功能衰竭。死亡者与存活者相比，肺动脉压力显著升高，动脉氧分压（PaO2）明显降低。47例患者（88．7％）使用传统的降压药治疗肺动脉高压，新型血管扩张药使用较少。结论不同结缔组织病间肺动脉高压的发生率有较大差异。结缔组织病继发肺动脉高压的主要表现是呼吸困难和肢端雷诺征。严重肺动脉高压将影响结缔组织病患者的生存率，肺动脉压力和PaO2的检测有利于判断预后。Objective To summarize the clinical characteristics of pulmonary arterial hyperten- sion （PAH） secondary to connective tissue disease （ CTD ）. Method Clinical data of 53 cases of PAH secondary to CTD admitted to the Huashan Hospital were investigated retrospectively. The classifications, clinical features, laboratory examinations, therapy and prognosis were collected. Results Among the 53 cases,46 females and 7 males, aged （43.9 ±13.8） years. Systemic lupus erythematosus was the most common disease. The most common clinical manifestation were dyspnea（ 84.9% ） and Raynaud syndrome （56.6%）. Antinuc]ear antibody, anti-nRNP antibody and anti-SSA antibody were higher than others. 7 patients（ 13.2% ） died during the follow-up, and the main cause of death was right heart failure. Com- pared to the survival,lower PaO2 and higher pulmonary artery systolic pressure（PASP） were found in the death group. As to PAH treatment traditional vasodilator agents （88.7%） were the first choice, and the new type were seldom uesd. Conclusion The incidence of PAH was different in every disease. Dyspnea and Raynaud syndrome were the most commom manifestations. PAH associated to CTD could be very se- vere, which would reduce the survival rate of the patients. The levels of PaO2 and PASP are useful to pre- dict the prognosis of PAH.

关 键 词：结缔组织病 肺动脉高压 

分 类 号：R593.2[医药卫生—内科学]