title: A cohort study of mortality predictors and characteristics of patients with combined pulmonary fibrosis and emphysema.
abstract: OBJECTIVE
  Our purpose was to assess the clinical data, predictors of mortality and acute exacerbation (AE) in combined pulmonary fibrosis and emphysema (CPFE) patients.
  
  
  METHODS
  Single-centre retrospective cohort study.
  
  
  METHODS
  Teaching hospital in Japan.
  
  
  METHODS
  We identified 93 CPFE patients with high-resolution computed tomographic (HRCT) through multidisciplinary discussion. Patients who had connective tissue disease, drug-associated interstitial lung disease and occupationally related interstitial lung disease, such as asbestosis and silicosis, were excluded.
  
  
  METHODS
  There were no interventions.
  
  
  METHODS
  Medical records and HRCT scans from January 2002 through December 2007 were reviewed retrospectively at our hospital. Ninety-three patients had CPFE.
  
  
  RESULTS
  The mean age of CPFE patients was 74 years. Idiopathic pulmonary fibrosis and non-specific interstitial pneumonia were observed as distinct HRCT patterns. Forty-two patients showed finger clubbing. Mean serum Krebs von den Lungen-6 (KL-6) and per cent predicted forced vital capacity (%FVC) were 1089 IU/l, 63.86%, respectively. Twenty-two patients developed AE during observation period. Baseline KL-6 was a strong predictor of AE (OR=1.0016, p=0.009). Finger clubbing (HR=2.2620, p=0.015) and per cent predicted forced expiratory volume in one second/%FVC more than 1.2 (HR=1.9259, p=0.048) were independent predictors of mortality in CPFE.
  
  
  CONCLUSIONS
  Baseline serum KL-6 was a useful predictor of AE (cut-off =1050, receiver operator characteristic curve: 0.7720), which occurred in 24% (22/93) of the CPFE patients. Finger clubbing and per cent predicted forced expiratory volume in one second/%FVC more than 1.2 were independent predictors of mortality.
authors.affiliation: Department of Respiratory Medicine, Okinawa Chubu Hospital, Uruma, Japan.
authors.forename: Tomoo
authors.initials: T
authors.lastname: Kishaba
authors.forename: Yousuke
authors.initials: Y
authors.lastname: Shimaoka
authors.forename: Hajime
authors.initials: H
authors.lastname: Fukuyama
authors.forename: Kyoko
authors.initials: K
authors.lastname: Yoshida
authors.forename: Maki
authors.initials: M
authors.lastname: Tanaka
authors.forename: Shin
authors.initials: S
authors.lastname: Yamashiro
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Tamaki