结缔组织病相关动脉性肺动脉高压临床特点分析

目的:分析结缔组织病相关动脉性肺动脉高压（CTD-PAH）患者的临床特点,提高对疾病的认识。方法:回顾性总结经右心导管（RHC）确诊的70例CTD-PAH患者的临床资料,分析其特征。结果:患者均为女性,平均年龄（43.8±13.9）岁,最常见的PAH症状为活动后气短、乏力。33例（47.1%）有雷诺现象。常见的基础疾病为系统性红斑狼疮（SLE,48.6%）及原发性干燥综合征（pSS,20%）。抗SSA抗体阳性率（54.9%）较高。69例（98.6%）超声心动图结果提示可能存在肺动脉高压（PH）,38例（54.9%）有心包积液。患者1年及3年的生存率为96%和93%。结论:多种CTD可继发PAH,以SLE及pSS多见。雷诺现象、抗SSA抗体及心包积液可能是CTD-PAH发病的危险因素。超声心动图是筛查CTD-PAH的有效检查。激素及免抑制剂治疗可能对于改善病情有积极作用。

Clinical features of connective tissue disease associated Pulmonary artery hypertension

Objective: To analyze the characteristics of patients with Connective tissue disease associated Pulmonary artery hypertension (CTD-PAH) patients for improving understanding of the disease. Methods: The 70 CTD-PAH patients diagnosed by right heart catheterization(RHC) were analyzed retrospectively. Results: All patients were female, age was (43.8±13.9)years.The common symptoms of PAHwere dyspneaand fatigue.The rate of Raynaud’s phenomenon was 47.1%. The main underlying disease were systemic lupus erythematosus(48.6%) and primary Sjogren’s syndrome(20%). The positive rate of anti-SSA antibody was 54.9%.The 98.6% of the echocardiographic resultssuggested there may bethe pulmonary hypertension.The 1-year and 3-year survival rates were 96% and 93%. Conclusion: Pulmonary artery hypertension can occur in many connective tissue diseases, SLE and pSS are common. Raynaud’s phenomenon, anti-SSA antibody and pericardial effusion may be risk factors of CTD-PAH.Echocardiography contributes to the screening of CTD-PAH. Glucocorticoid and immunosuppressive therapy may have a positive effect on improving the disease.