title: Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis.
abstract: BACKGROUND
  Several predictors of mortality in patients with idiopathic pulmonary fibrosis have been described; however, there is a need for a practical and accurate method of quantifying the prognosis of individual patients.
  
  
  OBJECTIVE
  Develop a practical mortality risk scoring system for patients with idiopathic pulmonary fibrosis.
  
  
  METHODS
  We used a Cox proportional hazards model and data from two clinical trials (n = 1,099) to identify independent predictors of 1-year mortality among patients with idiopathic pulmonary fibrosis. From the comprehensive model, an abbreviated clinical model comprised of only those predictors that are readily and reliably ascertained by clinicians was derived. Beta coefficients for each predictor were then used to develop a practical mortality risk scoring system.
  
  
  RESULTS
  Independent predictors of mortality included age, respiratory hospitalization, percent predicted FVC, 24-week change in FVC, percent predicted carbon monoxide diffusing capacity, 24-week change in percent predicted carbon monoxide diffusing capacity, and 24-week change in health-related quality of life. An abbreviated clinical model comprising only four predictors (age, respiratory hospitalization, percent predicted FVC, and 24-wk change in FVC), and the corresponding risk scoring system produced estimates of 1-year mortality risk consistent with observed data (9.9% vs. 9.7%; C statistic = 0.75; 95% confidence interval, 0.71–0.79).
  
  
  CONCLUSIONS
  The prognosis for patients with idiopathic pulmonary fibrosis may be accurately determined using four readily ascertainable predictors. Our simplified scoring system may be a valuable tool for determining prognosis and guiding clinical management. Additional research is needed to validate the applicability and accuracy of the scoring system.
authors: Imperial College, 1B Manresa Road, London, UK. ron@du-bois.co.uk
authors: Roland M
authors: RM
authors: du Bois
authors: Derek
authors: D
authors: Weycker
authors: Carlo
authors: C
authors: Albera
authors: Williamson Z
authors: WZ
authors: Bradford
authors: Ulrich
authors: U
authors: Costabel
authors: Alex
authors: A
authors: Kartashov
authors: Lisa
authors: L
authors: Lancaster
authors: Paul W
authors: PW
authors: Noble
authors: Ganesh
authors: G
authors: Raghu
authors: Steven A
authors: SA
authors: Sahn
authors: Javier
authors: J
authors: Szwarcberg
authors: Michiel
authors: M
authors: Thomeer
authors: Dominique
authors: D
authors: Valeyre
authors: Talmadge E
authors: TE
authors: King