@Article {Qian1800081，作者= {Qian，Junyan和Li，Mengtao和张，萧和王，钱和赵，九良，壮，壮，左佐，晓霞和张，苗家和朱，平和啊，张张，魏和郑，彝族，吴芳和李，杨，张，庄子，冯，刘，彝族，彝族，燕，萧光峰}，编辑= {和黄，C.和阳，X.和徐，D。和刘，Y。和郭，X.和王，H.和Lai，J.和Zhang，N.和Yang，K.和Liu，Y.和Dong，S.和Lei，Y.和Feng，Y.和李，Y。和周，Y。和王，Q.和Jia，J.和Han，Q.和Guo，L.和Chen，J.和Zhang，Y. and Liu, Y. and Xu, H. and Sun, Y. and Hao, Y. and Fan, Y. and Shu, Y.Q. and Lin, Z. and Qing, P.}, title = {Long-term prognosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study}, volume = {53}, number = {2}, elocation-id = {1800081}, year = {2019}, doi = {10.1183/13993003.00081-2018}, publisher = {European Respiratory Society}, abstract = {This study aimed to identify the long-term clinical outcomes and prognostic factors of patients with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) confirmed by right heart catheterisation.A multicentre prospective cohort of SLE-associated PAH was established. Baseline and follow-up records were collected. The primary end-point was death. The secondary exploratory end-point was treatment goal achievement (TGA), defined as an integrated outcome.In total, 310 patients were enrolled from 14 PAH centres. The 1-, 3- and 5-year survival rates were 92.1\%, 84.8\% and 72.9\%, respectively. The 1-, 3- and 5-year TGA rates were 31.5\%, 53.6\% and 62.7\%, respectively. Baseline serositis, 6-min walking distance \>380 m and cardiac index >=2.5 L{\textperiodcentered}min-1{\textperiodcentered}m-2 were identified as independent prognostic factors of TGA. Patients with baseline serositis were more likely to reach TGA after intensive immunosuppressive therapy. TGA was identified as a positive predictor of survival in patients with SLE-associated PAH.TGA was associated with long-term survival, which supports the treat-to-target strategy in SLE-associated PAH. Baseline heart function predicted both survival and treatment goal achievement in patients with SLE-associated PAH. Patients with serositis at baseline tended to benefit from intensive immunosuppressive therapy and have a better clinical outcome.The long-term prognosis of SLE-associated PAH is newly reported by a large multicentre cohort study http://ow.ly/FRaS30mNAZA}, issn = {0903-1936}, URL = {//www.qdcxjkg.com/content/53/2/1800081}, eprint = {//www.qdcxjkg.com/content/53/2/1800081.full.pdf}, journal = {European Respiratory Journal} }