Abstract

BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an overall poor prognosis. A simple-to-use staging system for IPF may improve prognostication, help guide management, and facilitate research.
OBJECTIVE
To develop a multidimensional prognostic staging system for IPF by using commonly measured clinical and physiologic variables.
DESIGN
A clinical prediction model was developed and validated by using retrospective data from 3 large, geographically distinct cohorts.
SETTING
Interstitial lung disease referral centers in California, Minnesota, and Italy.
PATIENTS
228 patients with IPF at the University of California, San Francisco (derivation cohort), and 330 patients at the Mayo Clinic and Morgagni-Pierantoni Hospital (validation cohort).
MEASUREMENTS
The primary outcome was mortality, treating transplantation as a competing risk. Model discrimination was assessed by the c-index, and calibration was assessed by comparing predicted and observed cumulative mortality at 1, 2, and 3 years.
RESULTS
Four variables were included in the final model: gender (G), age (A), and 2 lung physiology variables (P) (FVC and Dlco). A model using continuous predictors (GAP calculator) and a simple point-scoring system (GAP index) performed similarly in derivation (c-index of 70.8 and 69.3, respectively) and validation (c-index of 69.1 and 68.7, respectively). Three stages (stages I, II, and III) were identified based on the GAP index with 1-year mortality of 6%, 16%, and 39%, respectively. The GAP models performed similarly in pooled follow-up visits (c-index ≥71.9).
LIMITATION
Patients were drawn from academic centers and analyzed retrospectively.
CONCLUSION
The GAP models use commonly measured clinical and physiologic variables to predict mortality in patients with IPF.

Links

Publisher Full Text

Authors

Ley B, Ryerson CJ, Vittinghoff E, Ryu JH, Tomassetti S, Lee JS, Poletti V, Buccioli M, Elicker BM, Jones KD, King TE, Collard HR

Institution

Department of Medicine, University of California, San Francisco, 505 Parnassus Avenue, Box 0111, San Francisco, CA 94143, USA. brett.ley@ucsf.edu

Source

Annals of internal medicine 156:10 2012 May 15 pg 684-91

MeSH

Age Factors
Female
Humans
Idiopathic Pulmonary Fibrosis
Lung
Lung Transplantation
Male
Middle Aged
Models, Statistical
Prognosis
Pulmonary Diffusing Capacity
Retrospective Studies
Risk Assessment
Vital Capacity

Pub Type(s)

Journal Article
Multicenter Study
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

22586007