Associations of interstitial lung disease subtype and CT pattern with lung function and survival.

Jun 09, 2025

Experts: John S Kim,Janelle Pugashetti,Shwu-Fan Ma,Yong Huang,Anna J Podolanczuk,David A Lynch,Andrea Oh,Josyf Mychaleckyj,Ani Manichaikul,Stephen Rich,Felix Chua,Traci M Adams,Kevin R Flaherty,Joyce S Lee,Joseph A Lasky,Ganesh Raghu,Susan Murray,Catherine Spino,Fernando J Martinez,Imre Noth,Stephen M Humphries,Ayodeji Adegunsoye,Philip L Molyneaux,Justin M Oldham,Chad A Newton

Prior work suggests different interstitial lung diseases (ILDs) that share the radiological usual interstitial pneumonia (UIP) pattern have an overall worse prognosis. However, epidemiological data with longitudinal sampling and replication remains lacking.
Data was used from the Pulmonary Fibrosis Foundation Patient Registry (PFF-PR) (n=932) and a meta-cohort of ILD research studies (n=1579). Linear mixed-effects models and Cox proportional hazard models were used to determine forced vital capacity (FVC) slopes and 5-year transplant-free survival, respectively, by ILD diagnosis and UIP radiological pattern. Secondarily, we examined FVC and survival by diagnosis and radiological fibrosis quantified by data-driven texture analysis (DTA) in the PFF-PR. Models were adjusted for age, sex, smoking and antifibrotic and immunosuppression medication use.
The proportions of idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP) and connective tissue disease (CTD)-ILD were the following for PFF-PR (70%, 11%, 19%) and meta-cohort (21%, 32%, 47%). In the PFF-PR, CTD-ILD with UIP CT pattern was associated with slower FVC decline (-34.4 mL/year) compared with IPF (-158.4 mL/year) and longer transplant-free survival (HR 0.50, 95% CI 0.29 to 0.85). This was replicated in the meta cohort for FVC (-53.1 vs -185.9 mL/year, p<0.0001) and survival (HR 0.38, 95% CI 0.27 to 0.53). A similar pattern was seen using DTA to objectively categorise patients into higher and lower radiological fibrosis. Between IPF and FHP-UIP, FVC decline was not significantly different in the PFF-PR (-203.4 vs -158.4 mL/year, p=0.58) and meta-cohort (-124.0 vs -185.9 mL/year, p=0.25).
Even in the presence of a UIP CT pattern, there may still be differences in lung function over time and survival, particularly for CTD-ILD.

© Author(s) (or their employer(s)) 2025. No commercial re-use. See rights and permissions. Published by BMJ Group.

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ABOUT THE EXPERTS

John S Kim,Janelle Pugashetti,Shwu-Fan Ma,Yong Huang,Anna J Podolanczuk,David A Lynch,Andrea Oh,Josyf Mychaleckyj,Ani Manichaikul,Stephen Rich,Felix Chua,Traci M Adams,Kevin R Flaherty,Joyce S Lee,Joseph A Lasky,Ganesh Raghu,Susan Murray,Catherine Spino,Fernando J Martinez,Imre Noth,Stephen M Humphries,Ayodeji Adegunsoye,Philip L Molyneaux,Justin M Oldham,Chad A Newton

John S Kim

Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA jk6jb@uvahealth.org.

Janelle Pugashetti

Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA.

Shwu-Fan Ma

Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA.

Yong Huang

Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA.

Anna J Podolanczuk

Department of Medicine, Weill Cornell Medical College, New York, New York, USA.

David A Lynch

Radiology, National Jewish Health, Denver, Colorado, USA.

Andrea Oh

Department of Radiology, University of California Los Angeles, Los Angeles, California, USA.

Josyf Mychaleckyj

Department of Genome Sciences, University of Virginia School of Medicine, Charlottesville, Virginia, USA.

Ani Manichaikul

Department of Genome Sciences, University of Virginia School of Medicine, Charlottesville, Virginia, USA.

Stephen Rich

Department of Genome Sciences, University of Virginia School of Medicine, Charlottesville, Virginia, USA.

Felix Chua

Department of Respiratory Medicine, Royal Brompton Hospital, Guy’s and St. Thomas’s NHS Foundation Trust and National Heart and Lung Institute Imperial College London, London, UK.

Traci M Adams

Department of Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

Kevin R Flaherty

Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA.

Joyce S Lee

Department of Medicine, University of Colorado, Denver, Colorado, USA.

Joseph A Lasky

Medicine, Tulane University, New Olreans, Louisiana, USA.

Ganesh Raghu

University of Washington, Seattle, Washington, USA.

Susan Murray

Biostatistics, University of Michigan, Ann Arbor, Michigan, USA.

Catherine Spino

Biostatistics, University of Michigan, Ann Arbor, Michigan, USA.

Fernando J Martinez

Department of Medicine, University of Massachusetts Chan Medical School-UMass Memorial Health System, Worcester, Massachusetts, USA.

Imre Noth

Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA.

Stephen M Humphries

Radiology, National Jewish Health, Denver, Colorado, USA.

Ayodeji Adegunsoye

Pulmonary and Critical Care Medicine, University of Chicago, Chicago, Illinois, USA.

Philip L Molyneaux

Department of Respiratory Medicine, Royal Brompton Hospital, Guy’s and St. Thomas’s NHS Foundation Trust and National Heart and Lung Institute Imperial College London, London, UK.

Justin M Oldham

Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA.

Chad A Newton

Department of Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.