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Fibrotic Hypersensitivity Pneumonitis with Granulomas

Imaging findings

CT shows opacities extending from the upper to lower lung zones with a conspicuous absence of basilar subpleural fibrosis, traction bronchiectasis, or definite honeycombing, and areas of airway-centric fibrosis away from the pleural surfaces. The distribution is more upper-lung predominant and central with excess ground glass, favoring an alternative diagnosis over UIP. Surgical lung biopsy showed numerous loose non-necrotizing airway-centered granulomas, an interstitial granuloma, granulomatous airway remodeling with peribronchiolar metaplasia, scarred airways, alveolar septal expansion, and rare fibroblastic foci.

Key takeaways

Upper-lung and central-predominant fibrosis with excess ground glass and airway-centric fibrosis is best labeled as suggestive of an alternative diagnosis rather than UIP, and most such cases prove to be hypersensitivity pneumonitis or connective-tissue-disease-related interstitial pneumonia rather than IPF. Loose non-necrotizing airway-centered granulomas and peribronchiolar metaplasia on biopsy support fibrotic hypersensitivity pneumonitis, and the unclassifiable category remains useful because many of these patients ultimately reach a familial or connective-tissue-disease diagnosis.

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