• Daniel-Costin Marinescu, Cameron J Hague, Nestor L Muller, Darra Murphy, Andrew Churg, Joanne L Wright, Amna Al-Arnawoot, Ana-Maria Bilawich, Patrick Bourgouin, Gerard Cox, Celine Durand, Tracy Elliot, Jennifer Ellis, Jolene H Fisher, Derek Fladeland, Amanda Grant-Orser, Gillian C Goobie, Zachary Guenther, Ehsan Haider, Nathan Hambly, James Huynh, Kerri A Johannson, Geoffrey Karjala, Nasreen Khalil, Martin Kolb, Jonathon Leipsic, Stacey Lok, Sarah MacIsaac, Micheal McInnis, Helene Manganas, Veronica Marcoux, John Mayo, Julie Morisset, Ciaran Scallan, Tony Sedlic, Shane Shapera, Kelly Sun, Victoria Tan, Alyson W Wong, Boyang Zheng, and Christopher J Ryerson.
• Department of Medicine, University of British Columbia, Vancouver, BC, Canada; Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada. Electronic address: daniel.marinescu@vch.ca.
• Chest. 2023 Dec 1; 164 (6): 146614751466-1475.

BackgroundClinical practice guidelines separately describe radiologic patterns of usual interstitial pneumonia (UIP) and fibrotic hypersensitivity pneumonitis (fHP), without direction on whether or how to apply these approaches concurrently within a single patient.Research QuestionHow can we integrate guideline-defined radiologic patterns to diagnose interstitial lung disease (ILD) and what are the pitfalls associated with described patterns that require reassessment in future guidelines?Study Design And MethodsPatients from the Canadian Registry for Pulmonary Fibrosis underwent detailed reevaluation in standardized multidisciplinary discussion. CT scan features were quantified by chest radiologists masked to clinical data, and guideline-defined patterns were assigned. Clinical data then were provided to the radiologist and an ILD clinician, who jointly determined the leading diagnosis.ResultsClinical-radiologic diagnosis in 1,593 patients was idiopathic pulmonary fibrosis (IPF) in 26%, fHP in 12%, connective tissue disease-associated ILD (CTD-ILD) in 34%, idiopathic pneumonia with autoimmune features in 12%, and unclassifiable ILD in 10%. Typical and probable UIP patterns corresponded to a diagnosis of IPF in 66% and 57% of patients, respectively. Typical fHP pattern corresponded to an fHP clinical diagnosis in 65% of patients, whereas compatible fHP was nonspecific and associated with CTD-ILD or IPAF in 48% of patients. No pattern ruled out CTD-ILD. Gas trapping affecting > 5% of lung parenchyma on expiratory imaging was an important feature broadly separating compatible and typical fHP from other patterns (sensitivity, 0.77; specificity, 0.91).InterpretationAn integrated approach to guideline-defined UIP and fHP patterns is feasible and supports > 5% gas trapping as an important branch point. Typical or probable UIP and typical fHP patterns have moderate predictive values for a corresponding diagnosis of IPF and fHP, although occasionally confounded by CTD-ILD; compatible fHP is nonspecific.Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.

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