Other interstitial pneumonitis / fibrosis
Bronchiolocentric interstitial pneumonia
Reviewers: Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 24 September 2011, last major update September 2011
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● Unclear if distinct entity
● Uncommon disease resembling hypersensitivity pneumonitis, however, much more aggressive course
● 80% women, ages 40-50 years
● X-ray: interstitial and restrictive lung disease
● Poor prognosis - 33% dead after mean of 4 years (Mod Pathol 2002;15:1148)
● Similar pattern seen with rheumatoid arthritis, Sjogren’s syndrome, scleroderma, methotrexate and amiodarone (5-10% of patients)
● Centrilobular inflammation with peribronchiolar fibrosis and inflammation radiating into interstitium of distal acinus in a patchy fashion
● Inflammatory process begins at bronchovascular bundle, accompanied by centrilobular fibrosis and metaplasia
● Periphery of lobule shows relative sparing
● No granulomas, no honeycomb change
● Hypersensitivity pneumonitis: specific cause usually identifiable, less centrilobular fibrosis, usually has granulomas
● Non-specific interstitial pneumonia: by definition, excludes bronchiolocentric interstitial injury
End of Lung-nontumor > Other interstitial pneumonitis / fibrosis > Bronchiolocentric interstitial pneumonia
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