title: [The clinical characteristics of systemic sclerosis-related pulmonary arterial hypertension].
abstract: OBJECTIVE
  To study the clinical, cardiopulmonary functional and hemodynamic profiles of systemic sclerosis patients with pulmonary hypertension (SSc-PAH) compared with those of idiopathic pulmonary hypertension (IPAH).
  
  
  METHODS
  Patients diagnosed with SSc-PAH or IPAH by right heart catheterization were consecutively enrolled into the study between 2011 and 2013 in Peking Union Medical College Hospital (PUMCH). Cases with pulmonary hypertension related to other diseases were excluded. Demographic data, laboratory parameters, 6 minutes walk distance (6MWD), pulmonary function and hemodynamic variables at the time of diagnosis were collected and compared between the two groups.
  
  
  RESULTS
  A total of 20 SSc-PAH patients including 19 females and one male with age of (43.1 ± 12.2) years, and 18 IPAH patients including 16 females and 2 males with age of (38.4 ± 12.4) years were enrolled in this study. Subjects in both groups had similar mean pulmonary arterial pressure, cardiac index and pulmonary vascular resistance (PVR) when recruited. Compared with IPAH patients, SSc-PAH patients showed significantly decreased all parameters including forced vital capacity (FVC)% [(77.1 ± 13.2)% vs (88.6 ± 14.9)%, P = 0.026], diffusing capacity of the lung for carbon monoxide (DLCO)% [(46.2 ± 13.1) % vs (66.6 ± 13.3)%, P < 0.001], DLCO/alveolar ventilation (VA) [(55.1 ± 14.3)% vs (75.1 ± 11.5)%, P < 0.001], and 6MWD [(365.6 ± 85.1) m vs (454.3 ± 136.8) m, P = 0.034]. In subgroup analysis of SSc-PAH patients, elevated PVR (OR 2.122, 95%CI 1.093-4.119, P = 0.026) and decreased DLCO% (OR 0.916, 95%CI 0.842-0.996, P = 0.040) were independently associated with reduced 6MWD.
  
  
  CONCLUSIONS
  Under the similar hemodynamic condition, SSc-PAH patients had more severe restrictive ventilation dysfunction and diffusion capacity dysfunction. Decreased 6MWD in SSc-PAH patients was probably related to the impairment of pulmonary function.
authors.forename: Hui
authors.initials: H
authors.lastname: Wang
authors.affiliation: Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing 100032, China. Email: tianzhuangcn@sina.com.
authors.forename: Zhuang
authors.initials: Z
authors.lastname: Tian
authors.forename: Yongtai
authors.initials: Y
authors.lastname: Liu
authors.forename: Mengtao
authors.initials: M
authors.lastname: Li
authors.forename: Qian
authors.initials: Q
authors.lastname: Wang
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Zeng
authors.forename: Quan
authors.initials: Q
authors.lastname: Fang
mesh_terms: D000328:Adult; D000368:Aged; D006328:Cardiac Catheterization; D065627:Familial Primary Pulmonary Hypertension / Q000175:diagnosis*; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D016896:Treatment Outcome