A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis
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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.
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The record
- Venue
- Annals of Internal Medicine
- Topic
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Field
- Medicine
- Canadian institutions
- University of British Columbia
- Funders
- National Heart, Lung, and Blood Institute
- Keywords
- MedicineIdiopathic pulmonary fibrosisDLCOInternal medicineLung transplantationCohortInterstitial lung diseaseRetrospective cohort studyTransplantationSurgeryLungDiffusing capacityLung function
- Has abstract in OpenAlex
- yes