title: Histological analysis of vasculopathy associated with pulmonary hypertension in combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis or emphysema alone.
abstract: OBJECTIVE
  To evaluate pulmonary vasculopathy in an autopsy series of patients with combined pulmonary fibrosis and emphysema (CPFE), and compare these findings with those of patients with idiopathic pulmonary fibrosis (IPF) alone and emphysema alone.
  
  
  RESULTS
  We retrospectively analysed the clinical, radiological and pathological features of 26 patients with CPFE, 11 with IPF, and 23 with emphysema. We evaluated pulmonary vascular, venous-venular and arteriolar tissue changes in the fibrotic, emphysematous and relatively unaffected (preserved) areas by using the Heath-Edwards scoring system. We found moderate-to-severe vasculopathy in the CPFE group, but no significant differences in the fibrotic and emphysematous areas among the three groups. However, in the preserved area, the grading was significantly different among the three groups (P < 0.001), and vasculopathy in the CPFE group was the most severe. Although venous-venular and arteriolar changes in almost all fibrotic and emphysematous areas in the three groups showed no significant differences, there were significant differences in venous-venular (P = 0.004) and arteriolar (P < 0.001) changes in the preserved area among the three groups, which were most prevalent in the CPFE group. In the CPFE group, venous-venular changes and vasculopathy by Heath-Edwards grading were highest in the fibrotic area and lowest in the preserved area.
  
  
  CONCLUSIONS
  These results imply that pulmonary vasculopathy in patients with CPFE could occur in the whole lung tissue. This may explain the tendency for it to lead to the development of pulmonary hypertension in CPFE cases.
authors.affiliation: Department of Human Pathology, Tokyo Medical and Dental University Graduate School, Tokyo, Japan.|Department of Respiratory Medicine, Japanese Red Cross Medical Centre, Tokyo, Japan.
authors.forename: Nobuyasu
authors.identifier: http://orcid.org/0000-0002-1438-4880
authors.initials: N
authors.lastname: Awano
authors.affiliation: Department of Respiratory Medicine, Tokyo Metropolitan Hiroo Hospital, Tokyo, Japan.
authors.forename: Minoru
authors.initials: M
authors.lastname: Inomata
authors.affiliation: Department of Respiratory Medicine, Japanese Red Cross Medical Centre, Tokyo, Japan.
authors.forename: Soichiro
authors.initials: S
authors.lastname: Ikushima
authors.affiliation: Department of Radiology, Japanese Red Cross Medical Centre, Tokyo, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Yamada
authors.affiliation: Department of Radiology, Japanese Red Cross Medical Centre, Tokyo, Japan.
authors.forename: Masatoshi
authors.initials: M
authors.lastname: Hotta
authors.affiliation: Department of Radiology, Japanese Red Cross Medical Centre, Tokyo, Japan.
authors.forename: Shunji
authors.initials: S
authors.lastname: Tsukuda
authors.affiliation: Department of Pathology, Japanese Red Cross Medical Centre, Tokyo, Japan.
authors.forename: Toshio
authors.initials: T
authors.lastname: Kumasaka
authors.affiliation: Department of Pathology, Japanese Red Cross Medical Centre, Tokyo, Japan.
authors.forename: Tamiko
authors.initials: T
authors.lastname: Takemura
authors.affiliation: Department of Human Pathology, Tokyo Medical and Dental University Graduate School, Tokyo, Japan.
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Eishi
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000473:pathology*; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011656:Pulmonary Emphysema / Q000150:complications / Q000473:pathology*; D012189:Retrospective Studies; D014652:Vascular Diseases / Q000473:pathology*
keywords: combined pulmonary fibrosis and emphysema; emphysema; idiopathic pulmonary fibrosis; pulmonary hypertension; vasculopathy