Bronchiolocentric Fibrosis in Connective Tissue Disease
Imaging findings
CT in a patient with connective tissue disease shows atypical fibrotic changes with prominent cystic spaces, basal predominance, and subpleural sparing. Pathology demonstrated bronchiolocentric fibroblastic foci and airway obliteration representing bronchiolocentric fibrosis.
Key takeaways
Interstitial lung disease in patients with connective tissue disease can present with an atypical, bronchiolocentric pattern of fibrosis rather than subpleural-predominant UIP, showing prominent airway-centered cysts and fibroblastic foci on biopsy.
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