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04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pathologic patterns and survival in chronic hypersensitivity pneumonitis.
Andrew Churg1, Don D Sin, Douglas Everett
1Department of Pathology, University of British Columbia, Vancouver, BC, Canada. achurg@interchange.ubc.ca
The American Journal of Surgical Pathology
|October 8, 2009
Summary
This study on hypersensitivity pneumonitis (HP) found that fibrosis generally indicates a poor prognosis. However, pure peribronchiolar fibrosis may offer longer survival compared to UIP-like or fibrotic NSIP patterns.
Area of Science:
- Pulmonary Pathology
- Interstitial Lung Diseases
- Immunology
Background:
- Hypersensitivity pneumonitis (HP) is an immune-mediated lung disease.
- Distinguishing between subacute and chronic HP is crucial for prognosis.
- Pathologic patterns in HP can mimic other interstitial lung diseases.
Purpose of the Study:
- To investigate the relationship between pathologic patterns and prognosis in hypersensitivity pneumonitis.
- To characterize the histopathologic features of subacute and chronic HP.
- To compare survival outcomes based on distinct fibrotic patterns.
Main Methods:
- Analysis of 49 HP cases: 24 subacute (cellular, nonfibrotic) and 25 chronic (fibrotic).
- Histopathologic evaluation for patterns including bronchiolocentric interstitial pneumonia, cellular nonspecific interstitial pneumonia (NSIP)-like, usual interstitial pneumonia (UIP)-like, and peribronchiolar fibrosis.
- Assessment of giant cells, granulomas, and Schaumann bodies.
- Correlation of identified patterns with patient survival data.
Main Results:
- Subacute HP cases showed bronchiolocentric interstitial pneumonia (79%) or cellular NSIP-like patterns (21%).
- Chronic HP cases frequently exhibited UIP-like patterns (72%) with prominent peribronchiolar fibrosis.
- Presence of fibrosis was associated with poor prognosis; pure peribronchiolar fibrosis showed a median survival of 11.3 years, while UIP-like and fibrotic NSIP-like patterns had significantly shorter survival (2.8 and 2.1 years, respectively).
- Areas of subacute HP were found in 12 cases with UIP-like patterns.
Conclusions:
- Fibrosis in hypersensitivity pneumonitis generally correlates with a poor prognosis.
- Pure peribronchiolar fibrosis may indicate a better survival outcome than UIP-like or fibrotic NSIP-like patterns.
- While chronic HP often has distinct features, some cases are pathologically indistinguishable from idiopathic UIP.
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