TY - JOUR T1 - Connective tissue disease-associated pulmonary arterial hypertension: “Beijing style” JF - European Respiratory Journal JO - Eur Respir J SP - 839 LP - 841 DO - 10.1183/09031936.00090814 VL - 44 IS - 4 AU - Lorinda Chung AU - Steven M. Kawut Y1 - 2014/10/01 UR - http://erj.ersjournals.com/content/44/4/839.abstract N2 - Patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) experience poorer outcomes than those with idiopathic PAH (IPAH) [1–6]. In the USA, patients with systemic sclerosis (SSc) are most commonly affected by CTD-APAH and have the worst survival [1]. Similarly, 74% of a large cohort with CTD-APAH in the UK had SSc, and these patients had poorer survival than those with systemic lupus erythematosus (SLE) or polymyositis/dermatomyositis [7].Few published studies have described the clinical features and outcomes of patients with CTD-APAH in Asia. Chung et al. [8] found that Korean patients with SLE-pulmonary hypertension had much worse survival than those with IPAH, with 1- and 5-year survival rates of 51% and 17% for SLE-pulmonary hypertension compared with 77% and 68% for IPAH. A more recent study of 70 Japanese patients with CTD-APAH found that mixed connective tissue disease (MCTD) was the most common underlying CTD (43%), followed by SLE (29%), SSc (19%) and primary Sjogren’s syndrome (10%) [9]. This study found no significant difference in survival among the different CTD-APAH subgroups, but did find improved survival rates in those diagnosed more recently.In this issue of the European Respiratory Journal, Hao et al. [10] describe the clinical features and outcomes of 129 Chinese patients with CTD-APAH confirmed by right heart catheterisation between July 2006 and May 2011. It is known that the prevalence of SLE in the Chinese population is much higher than in Caucasians [11, 12], while SSc is more common in Caucasians than in the Chinese population [13]. Consistent with this epidemiology, SLE was the … ER -