A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis

作者：Brett Ley, Christopher J. Ryerson, Eric Vittinghoff, Jay Hoon Ryu, Sara Tomassetti, Joyce Lee, Venerino E. Poletti, Matteo Buccioli, Brett M. Elicker, Kirk D. Jones, Talmadge E. King, Harold R. Collard · 发表于：Annals of Internal Medicine · 年份：2012 · DOI：10.7326/0003-4819-156-10-201205150-00004 · 被引用次数：1349 · 研究领域：Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis、Systemic Sclerosis and Related Diseases、Transplantation: Methods and Outcomes

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 mortal...