title: Multi-institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases.
abstract: OBJECTIVE
  Pulmonary hypertension (PH) is often associated with respiratory diseases, but only a small number of patients present with severe PH defined as mean pulmonary arterial pressure ≥ 35 mm Hg. We here conducted a multicenter, retrospective study of patients with severe PH associated with respiratory diseases (R-PH) to reveal their demographics, treatment, prognosis and determinants of prognosis.
  
  
  METHODS
  From 101 patients with severe R-PH collected by postal survey at the first stage, 70 patients with four major diseases (chronic obstructive pulmonary disease (COPD), combined pulmonary fibrosis with emphysema (CPFE), interstitial pneumonia associated with connective tissue disease (CTD-IP), interstitial pneumonia (IP)) and normal pulmonary arterial wedge pressure were studied for clinical characteristics, treatment and prognosis.
  
  
  RESULTS
  Three-year survival rates were 50% for COPD (n = 18), 35.7% for IP (n = 19) and 68.1% for CTD-IP (n = 20), and the 2-year survival rate for CPFE (n = 13) was only 22.6%. Eighty-one per cent of patients had been treated with pharmacotherapy specific for pulmonary arterial hypertension. Those patients who had received phosphodiesterase-5 inhibitors (PDE-5I) displayed significantly better survival from the date of diagnosis than those who had not (3-year survival: 61.8% vs 20.0% P < 0.0001), especially in the IP, CTD-IP and CPFE groups. Multivariate analysis also revealed that treatment with PDE-5I was a positive prognostic factor.
  
  
  CONCLUSIONS
  We here demonstrated the dismal prognosis of patients with severe R-PH. The remarkably better survival in those patients who had received PDE-5I warrants and facilitates future prospective randomized studies in this particular population.
authors.affiliation: Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan.|Department of Advanced Medicine in Pulmonary Hypertension, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Tanabe
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: First Department of Medicine, Hokkaido University Hospital, Sapporo, Japan.
authors.forename: Ichizo
authors.initials: I
authors.lastname: Tsujino
authors.affiliation: Department of Medicine, Tokyo Saiseikai Central Hospital, Tokyo, Japan.
authors.forename: Fumio
authors.initials: F
authors.lastname: Sakamaki
authors.affiliation: Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Japan.
authors.forename: Noriaki
authors.initials: N
authors.lastname: Emoto
authors.affiliation: Second Department of Internal Medicine, Nara Medical University, Kashihara, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kimura
authors.affiliation: First Department of Medicine, Obihiro-Kosei General Hospital, Obihiro, Japan.
authors.forename: Kei
authors.initials: K
authors.lastname: Takamura
authors.affiliation: First Department of Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Hanaoka
authors.affiliation: First Department of Medicine, Hokkaido University Hospital, Sapporo, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Nishimura
authors.affiliation: Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Koichiro
authors.initials: K
authors.lastname: Tatsumi
mesh_terms: D000368:Aged; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D007564:Japan / Q000453:epidemiology; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D012140:Respiratory Tract Diseases / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate
chemical_list: D058986:Phosphodiesterase 5 Inhibitors
keywords: chronic obstructive pulmonary disease; clinical respiratory medicine; nitric oxide; pulmonary circulation and pulmonary hypertension; pulmonary fibrosis