title: Progression of mean pulmonary arterial pressure in idiopathic pulmonary fibrosis with mild to moderate restriction.
abstract: Elevation of mean pulmonary arterial pressure (MPAP) is associated with poor prognosis in patients with idiopathic pulmonary fibrosis (IPF), yet the progression of MPAP in patients with IPF has not been sufficiently elucidated. We evaluated serial changes in MPAP and its determinants in patients with IPF with mild to moderate restriction.
  
  
  
  We retrospectively reviewed patients with IPF who underwent initial evaluations including right heart catheterization (RHC) in our institute from May 2007 to December 2013 with follow-up RHC at least 1 year later. Patients with forced vital capacity (FVC) < 50% predicted or those with pulmonary artery wedge pressure >15 mm Hg were excluded.
  
  
  
  A total of 95 patients were included. Median follow-up time of second RHC was 1.8 years. MPAP increased significantly at follow-up (from 16.8 to 20.2 mm Hg; P < 0.001), and annual change in MPAP (ΔMPAP) was 1.8 mm Hg/year. In multiple regression analysis, the lowest oxygen saturation (SpO2) at 6-min walk test (6MWT) was an independent predictor of ΔMPAP. When adjusted for age, sex, baseline MPAP and FVC % predicted, ΔMPAP was a significant predictor of mortality (hazard ratio: 1.21; P = 0.001).
  
  
  
  ΔMPAP was significantly associated with desaturation in the 6MWT, and with increased mortality in patients with IPF with mild to moderate restriction.
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Teramachi
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Taniguchi
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kondoh
authors.affiliation: Center for Advanced Medicine and Clinical Research, Nagoya University Hospital, Nagoya, Japan.
authors.forename: Masahiko
authors.initials: M
authors.lastname: Ando
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Kimura
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
authors.forename: Kensuke
authors.initials: K
authors.lastname: Kataoka
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Suzuki
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan.
authors.forename: Taiki
authors.initials: T
authors.lastname: Furukawa
authors.affiliation: Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Sakamoto
authors.affiliation: Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Hasegawa
mesh_terms: D000368:Aged; D062186:Arterial Pressure; D006328:Cardiac Catheterization; D018450:Disease Progression; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D011669:Pulmonary Wedge Pressure; D012189:Retrospective Studies; D014797:Vital Capacity
keywords: idiopathic pulmonary fibrosis; interstitial lung disease; pulmonary arterial pressure; pulmonary hypertension