title: An autopsy study of combined pulmonary fibrosis and emphysema: correlations among clinical, radiological, and pathological features.
abstract: BACKGROUND
  Clinical evaluation to differentiate the characteristic features of pulmonary fibrosis and emphysema is often difficult in patients with combined pulmonary fibrosis and emphysema (CPFE), but diagnosis of pulmonary fibrosis is important for evaluating treatment options and the risk of acute exacerbation of interstitial pneumonia of such patients. As far as we know, it is the first report describing a correlation among clinical, radiological, and whole-lung pathological features in an autopsy cases of CPFE patients.
  
  
  METHODS
  Experts retrospectively reviewed the clinical charts and examined chest computed tomography (CT) images and pathological findings of an autopsy series of 22 CPFE patients, and compared these with findings from 8 idiopathic pulmonary fibrosis (IPF) patients and 17 emphysema-alone patients.
  
  
  RESULTS
  All patients had a history of heavy smoking. Forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC%) was significantly lower in the emphysema-alone group than the CPFE and IPF-alone groups. The percent predicted diffusing capacity of the lung for carbon monoxide (DLCO%) was significantly lower in the CPFE group than the IPF- and emphysema-alone groups. Usual interstitial pneumonia (UIP) pattern was observed radiologically in 15 (68.2%) CPFE and 8 (100%) IPF-alone patients and was pathologically observed in all patients from both groups. Pathologically thick-cystic lesions involving one or more acini with dense wall fibrosis and occasional fibroblastic foci surrounded by honeycombing and normal alveoli were confirmed by post-mortem observation as thick-walled cystic lesions (TWCLs). Emphysematous destruction and enlargement of membranous and respiratory bronchioles with fibrosis were observed in the TWCLs. The cystic lesions were always larger than the cysts of honeycombing. The prevalence of both radiological and pathological TWCLs was 72.7% among CPFE patients, but no such lesions were observed in patients with IPF or emphysema alone (p=0.001). The extent of emphysema in CPFE patients with TWCLs was greater than that in patients without such lesions. Honeycombing with emphysema was also observed in 11 CPFE patients.
  
  
  CONCLUSIONS
  TWCLs were only observed in the CPFE patients. They were classified as lesions with coexistent fibrosing interstitial pneumonia and emphysema, and should be considered an important pathological and radiological feature of CPFE.
authors: Department of Respiratory Medicine, Japanese Red Cross Medical Centre, 4-1-22 Hiroo, Shibuyaku, Tokyo 150-8953, Japan. inomataminoru@nms.ac.jp.
authors: Minoru
authors: M
authors: Inomata
authors: Soichiro
authors: S
authors: Ikushima
authors: Nobuyasu
authors: N
authors: Awano
authors: Keisuke
authors: K
authors: Kondoh
authors: Kohta
authors: K
authors: Satake
authors: Masahiro
authors: M
authors: Masuo
authors: Yuji
authors: Y
authors: Kusunoki
authors: Atsuko
authors: A
authors: Moriya
authors: Hiroyuki
authors: H
authors: Kamiya
authors: Tsunehiro
authors: T
authors: Ando
authors: Noriyo
authors: N
authors: Yanagawa
authors: Toshio
authors: T
authors: Kumasaka
authors: Takashi
authors: T
authors: Ogura
authors: Fumikazu
authors: F
authors: Sakai
authors: Arata
authors: A
authors: Azuma
authors: Akihiko
authors: A
authors: Gemma
authors: Tamiko
authors: T
authors: Takemura
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001344:Autopsy; D002248:Carbon Monoxide; D003560:Cysts / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008297:Male; D008875:Middle Aged; D011653:Pulmonary Diffusing Capacity; D011656:Pulmonary Emphysema / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology*; D011658:Pulmonary Fibrosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity
chemical_list: D002248:Carbon Monoxide