肺动脉高压与原发性Sjögren综合征相关:一个多中心队列研究从中国摩根富林明-欧洲呼吸杂志》乔和J - 10.1183/13993003.02157 -2019欧元六世- 56 - 5 SP - 1902157 AU - Wang Jieying AU - Li Mengtao AU -王,钱非盟-张小盟——钱,Junyan盟——赵Jiuliang盟——徐董盟——田,壮族非盟-魏,魏盟-左,庐山逍夏盟,张Miaojia盟——朱,平盟——你们双盟——张魏盟——郑,易盟——气、五方非盟- Li Yang盟——张Zhuoli AU -叮,冯盟,顾Jieruo盟——刘原发性Sjögren’s综合征(Primary Sjögren’s syndrome, pSS)是肺动脉高压(pulmonary arterial hypertension, PAH)的重要病因，目前对其研究尚不充分，值得重视。本研究旨在探讨pSS-PAH的临床特点、危险因素、预后及风险评估。方法建立经右心导管诊断的pSS-PAH多中心队列。以pss -非PAH患者为对照组进行病例对照研究，以确定PAH的危险因素。在队列研究中，计算生存率，并在基线和随访时进行风险评估。结果共纳入103例pSS-PAH患者，526例pss -非pah患者作为对照。抗ssb (p<0.001, OR 4.095)和抗u1rnp抗体(p<0.001, OR 29.518)、pSS发病年龄(p<0.001, OR 0.651)和角膜染色阳性(p=0.003, OR 0.409)的存在被确定为PAH的独立危险因素。1、3、5年生存率分别为94.0%、88.8%、79.0%。心脏指数(p=0.010，风险比(HR) 0.161)、肺血管阻力(p=0.016，风险比1.105)和Sjögren综合征疾病损害指数(p=0.006，风险比1.570)被确定为pSS-PAH患者死亡的潜在预测因素。在基线(p=0.002)和随访(p= 0.0001)时，低风险类别患者的长期预后得到改善。Conclusion The routine screening of PAH is suggested in pSS patients with early onset and positivity for anti-SSB or anti-U1RNP antibodies. Patient prognosis might be improved by improving reserved cardiopulmonary function, by achieving a damage-free state and especially by achieving low-risk category, which supports the treat-to-target strategy for pSS-PAH.The prognosis of primary Sjögren's syndrome-associated pulmonary arterial hypertension might be improved by improving reserved cardiopulmonary function, by achieving a damage-free state and especially by achieving low-risk category https://bit.ly/3h7mZ9h ER -