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Updated: Apr 28, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Idiopathic pulmonary fibrosis: CT and risk of death
Brett Ley1, Brett M Elicker, Thomas E Hartman
1From the Departments of Medicine (B.L., J.S.L., T.E.K., H.R.C.), Radiology (B.M.E.), Epidemiology and Biostatistics (E.V.), and Pathology (K.D.J.), University of California-San Francisco, 505 Parnassus Ave, Box 0111, San Francisco, CA 94143; Departments of Radiology (T.E.H.) and Medicine (J.H.R.), Mayo Clinic, Rochester, Minn; Department of Medicine, University of British Columbia, Vancouver, BC, Canada (C.J.R.); and Department of Respiratory Medicine, University of Southampton, Southampton, England (L.R.).
Purpose:
To investigate the prognostic value of quantitative computed tomographic (CT) scoring for the extent of fibrosis or emphysema in the context of a clinical model that includes the gender, age, and physiology ( GAP gender, age, and physiology model) of the patient.
Materials And Methods:
Study cohorts were approved by local institutional review boards, and all patients provided written consent. This was a retrospective cohort study that included 348 patients (246 men, 102 women; mean age, 69 years ± 9) with idiopathic pulmonary fibrosis from two institutions. Fibrosis and emphysema visual scores were independently determined by two radiologists. Models were based on competing risks regression for death and were evaluated by using the C index and reclassification improvement.
Results:
The CT- GAP gender, age, and physiology model (a modification of the original GAP gender, age, and physiology model that replaces diffusion capacity of carbon monoxide with CT fibrosis score) had accuracy comparable to that of the original GAP gender, age, and physiology model, with a C index of 70.3 (95% confidence interval: 66.4, 74.0); difference in C index compared with the GAP gender, age, and physiology model of -0.4 (95% confidence interval: -2.2, 3.4). The performance of the original GAP gender, age, and physiology model did not change significantly with the simple addition of fibrosis score, with a change in C index of 0.0 (95% confidence interval: -1.8, 0.5) or of emphysema score, with a change in C index of 0.0 [95% confidence interval: -1.3, 0.4]).
Conclusion:
CT fibrosis score can replace diffusion capacity of carbon monoxide test results in a modified GAP gender, age, and physiology model (the CT- GAP gender, age, and physiology model) with comparable performance. This may be a useful alternative model in situations where CT scoring is more reliable and available than diffusion capacity of carbon monoxide.
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