Outcomes of a Typical Fibrotic Hypersensitivity Pneumonitis Pattern on Chest Computed Tomography
- PMID: 40505139
- DOI: 10.1164/rccm.202411-2215OC
Outcomes of a Typical Fibrotic Hypersensitivity Pneumonitis Pattern on Chest Computed Tomography
Abstract
Background: Guidelines have defined a "typical hypersensitivity pneumonitis (HP)" imaging pattern for fibrotic HP (fHP); however, the frequency, characteristics, and outcomes of different multidisciplinary diagnoses within this pattern are unknown.
Methods: Patients with a typical fHP pattern on chest computed tomography (CT) were identified from a prospective registry. Multidisciplinary diagnoses were established by consensus during a research-dedicated standardized multidisciplinary discussion of all available data. Pre-specified diagnostic categories of interest included fHP with an exposure identified, fHP without an exposure identified, and connective tissue disease-associated interstitial lung disease (CTD-ILD), with each diagnosis defined by >50% likelihood after this structured multidisciplinary discussion. Clinical and radiological features and outcomes were compared across multidisciplinary diagnoses.
Results: Of 164 patients with a CT pattern of typical fHP, 49 had a multidisciplinary diagnosis of fHP with a probable or possible exposure identified (30%), 56 had fHP without an exposure (34%), 36 had a CTD-ILD (22%), and 23 had another multidisciplinary diagnosis (14%). Clinical and CT features differed across multidisciplinary diagnoses. Lung function decline and time to death or transplant were worse in fHP without a probable or possible exposure. Positive autoimmune serologies or a new rheumatologist-confirmed CTD diagnosis developed in 14% of patients with fHP without an exposure identified during follow-up.
Conclusion: Patients with a typical fHP pattern on chest CT frequently have non-HP diagnoses (most often CTD-ILD), have differences in baseline characteristics and disease behavior across multidisciplinary diagnoses, and more frequently develop features of CTD during follow-up when an initial HP exposure is not identified.
Keywords: bronchiolocentric interstitial pneumonia; computed tomography; hypersensitivity pneumonitis; outcomes; pulmonary fibrosis.
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