Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases  

作　　者：Bo Jiang Chun Wang Yansheng Jin Guanjun Guo Kangxing Zhou Xuebing Feng Genhong Yao 

机构地区：[1]Department of Rheumatology and Immunology,The Affiliated Drum Tower Hospital of Nanjing University Medical School,Nanjing,Jiangsu,China [2]Department of Rheumatology and Immunology,Wuzhong People's Hospital,Suzhou,Jiangsu,China [3]Department of Cardiac Ultrasound,The Affiliated Drum Tower Hospital of Nanjing University Medical School,Nanjing,Jiangsu,China

出　　处：《Rheumatology & Autoimmunity》2023年第3期157-165,共9页风湿病与自身免疫（英文）

基　　金：PANDA Project 2018-Clinical Research Special Fund of China Foundation for International Medical Exchange,Grant/Award Number:Z-2014-06-2-1863;Nanjing Science and Technology Development Plan,Grant/Award Number:201715021;Clinical Research Special fund of Nanjing Drum Tower Hospital,Grant/Award Number:2022-LCYJMS-39;Chinese Hospital Reform and Development Research Institute Project of Nanjing University,Grant/Award Number:NDYG2022047。

摘　　要：Background:Pulmonary arterial hypertension(PAH)associated with connective tissue diseases(CTD)(CTD-PAH)remains a difficult challenge in clinical practice.We aimed to evaluate the effects of targeted vasodilators in patients with severe CTD-PAH.Methods:The data of 53 patients with severe CTD-PAH hospitalized at the Department of Rheumatology and Immunology,The Affiliated Drum Tower Hospital of Nanjing University Medical School,were retrospectively reviewed.Patients were followed up for an average of 2 years to track their outcomes.The efficacy of treatment and the survival rate of patients with severe CTD-PAH were determined.Results:Among the causes of severe CTD-PAH,systemic lupus erythematosus(SLE)was the most common(39.6%),and the age at onset in patients with SLE-PAH was younger than that of patients with other CTD.Bosentan was more effective than sildenafil in reducing pulmonary artery pressure,improving cardiac function,and increasing survival time.Combination therapy with targeted vasodilators significantly improved the prognosis of patients with severe CTD-PAH compared with monotherapy.Conclusions:Patients with severe CTD-PAH should be treated early with targeted vasodilators.In this study,bosentan was superior to sildenafil.Combined treatment might be an option for severe CTD-PAH.

关 键 词：connective tissue disease pulmonary arterial hypertension systemic lupus erythematosus targeted vasodilator 

分 类 号：R73[医药卫生—肿瘤]