结缔组织病相关动脉性肺动脉高压临床特点分析  

Clinical features of connective tissue disease associated Pulmonary artery hypertension

作　　者：王慧[1] 张娜[1] 赵音 杨振文[2] 曹洁[3] 董丽霞[3] 魏蔚[1] WANG Hui;ZHANG Na;ZHAO Yin;YANG Zhen-wen;CAO Jie;DONG Li-xia;WEI Wei(Department of Rheumatology and Immunology,Tianjin Medical University,Tianjin 300052,China;Department of Cardiology,Tianjin Medical University,Tianjin 300052,China;Department of Respiration,General Hospital,Tianjin Medical University,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院风湿免疫科,天津300052 [2]天津医科大学总医院心脏科,天津300052 [3]天津医科大学总医院呼吸科,天津300052

出　　处：《天津医科大学学报》2020年第1期32-34,共3页Journal of Tianjin Medical University

摘　　要：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。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.

关 键 词：结缔组织病 动脉性肺动脉高压 右心导管 超声心动图 

分 类 号：R593.2[医药卫生—内科学]