Pulmonary arterial hypertension (PAH) is a clinicopathological syndrome caused by the increase of pulmonary artery, and it is the most serious complication of connective tissue disease (CTD). In recent years, a lot of progress has been made in the diagnosis, treatment and evaluation of PAH. Chinese Rheumatology Association formulated this recommendation on the basis of current experience and guidelines, in order to promote early screening, early diagnosis and early intervention of CTD-PAH, as well as patient follow-up and management, to improve the prognosis of CTD-PAH patients.
肺动脉高压（PAH）是多种原因导致肺动脉压升高而伴发的一系列临床病理综合征，PAH亦是结缔组织病（CTD）最严重的并发症。近年来有关PAH的诊治及评估有了很大进步，为提高风湿科医生对CTD相关PAH的认识与诊治水平，中华医学会风湿病学分会在目前国内外经验和指南的基础上，制定了本规范，旨在指导CTD相关PAH早期筛查、早期诊断和早期干预，以及如何进行患者随访与管理，以改善CTD相关PAH患者的长期预后。.