Integration and Application of Radiologic Patterns From Clinical Practice Guidelines on Idiopathic Pulmonary Fibrosis and Fibrotic Hypersensitivity Pneumonitis Journal Articles uri icon

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abstract

  • BACKGROUND: Clinical 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 QUESTION: How 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 METHODS: Patients 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. RESULTS: Clinical-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). INTERPRETATION: An 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.

authors

  • Marinescu, Daniel-Costin
  • Hague, Cameron J
  • Muller, Nestor L
  • Murphy, Darra
  • Churg, Andrew
  • Wright, Joanne L
  • Al-Arnawoot, Amna
  • Bilawich, Ana-Maria
  • Bourgouin, Patrick
  • Cox, Gerard
  • Durand, Celine
  • Elliot, Tracy
  • Ellis, Jennifer
  • Fisher, Jolene H
  • Fladeland, Derek
  • Grant-Orser, Amanda
  • Goobie, Gillian C
  • Guenther, Zachary
  • Haider, Ehsan
  • Hambly, Nathan
  • Huynh, James
  • Johannson, Kerri A
  • Karjala, Geoffrey
  • Khalil, Nasreen
  • Kolb, Martin Rainer
  • Leipsic, Jonathon
  • Lok, Stacey
  • MacIsaac, Sarah
  • McInnis, Micheal
  • Manganas, Helene
  • Marcoux, Veronica
  • Mayo, John
  • Morisset, Julie
  • Scallan, Ciaran Joseph
  • Sedlic, Tony
  • Shapera, Shane
  • Sun, Kelly
  • Tan, Victoria
  • Wong, Alyson W
  • Zheng, Boyang
  • Ryerson, Christopher J

publication date

  • December 2023

published in