title: Significance of pulmonary arterial pressure as a prognostic indicator in lung-dominant connective tissue disease.
abstract: BACKGROUND
  Lung-dominant connective tissue disease (LD-CTD) is a new concept for classifying the subset of patients with interstitial pneumonia who have clinical features suggesting an associated CTD, but whose features fall short of a clear diagnosis of CTD under the current rheumatologic classification systems. The impact of mean pulmonary arterial pressure (MPAP) in LD-CTD has not been sufficiently elucidated.
  
  
  OBJECTIVE
  To evaluate the survival impact of MPAP measured during the initial evaluation in patients with LD-CTD.
  
  
  METHODS
  We retrospectively analyzed the initial evaluation data of 100 LD-CTD patients undergoing pulmonary function test, 6-min walk test (6MWT), and right heart catheterization (RHC).
  
  
  RESULTS
  The mean MPAP was 16.2±4.4 mm Hg, and 18 patients had MPAP≥20 mm Hg. A univariate Cox proportional hazard model showed that MPAP and several variables have a statistically significant impact on survival. With stepwise, multivariate Cox proportional analysis, MPAP (HR  = 1.293; 95% CI 1.130-1.480; p<0.001) and mean forced vital capacity (FVC) % predicted (HR = 0.958; 95% CI 0.930-0.986; p = 0.004) were shown to be independent determinants of survival.
  
  
  CONCLUSIONS
  Higher MPAP and lower %FVC at the initial evaluation were significant independent prognostic factors of LD-CTD. MPAP evaluation provides additional information of disease status and will help physicians to predict mortality in LD-CTD.
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Suzuki
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Taniguchi
authors.affiliation: Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
authors.forename: Naohiro
authors.initials: N
authors.lastname: Watanabe
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kondoh
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Kimura
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Kensuke
authors.initials: K
authors.lastname: Kataoka
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Matsuda
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Toshiki
authors.initials: T
authors.lastname: Yokoyama
authors.affiliation: Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Sakamoto
authors.affiliation: Department of Respiratory Medicine and Allergology, Kinki University Faculty of Medicine, Osaka-sayama, Osaka, Japan.
authors.forename: Osamu
authors.initials: O
authors.lastname: Nishiyama
authors.affiliation: Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Hasegawa
mesh_terms: D000368:Aged; D062186:Arterial Pressure; D006328:Cardiac Catheterization; D003240:Connective Tissue Diseases / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D008168:Lung / Q000503:physiopathology*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D011651:Pulmonary Artery / Q000503:physiopathology*; D012189:Retrospective Studies; D016019:Survival Analysis; D014797:Vital Capacity; D016138:Walking