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Original Research |

Pathological Findings and Prognosis in a Large Prospective Cohort of Chronic Hypersensitivity Pneumonitis

Ping Wang, MD; Kirk D. Jones, MD; Anatoly Urisman, MD; Brett M. Elicker, MD; Thomas Urbania, MD; Kerri A. Johannson, MD; Deborah Assayag, MD; Joyce Lee, MD; Paul J. Wolters, MD; Harold R. Collard, MD; Laura L. Koth, MD
Author and Funding Information

Summary Conflict of Interest Statements: There are no conflicts for the following authors: P. Wang, K.D. Jones, A. Urisman, B.M. Elicker, T. Urbania, Joyce Lee, P. Wolters, H.R. Collard, K. A. Johannson, D. Assayag, and L.L. Koth

Funded by: Departmental sources and the Nina Ireland Program for Lung Health

Notification of prior abstract publication/presentation: Preliminary results of this study were presented as an abstract at the American Thoracic Society Annual Meeting in San Diego, CA in May 2014 (A6402).

1Department of Pulmonary Medicine, Peking Union Medical College Hospital , Chinese Academy of Medical Science &Peking Union Medical College, Beijing, China

2Department of Medicine

3Pathology, University of California San Francisco, CA, 94143

4Radiology, University of California San Francisco, CA, 94143

Corresponding author: Laura L. Koth, Box 0111, 505 Parnassus Avenue, San Francisco, CA 94143.


Copyright 2017, . All Rights Reserved.


Chest. 2017. doi:10.1016/j.chest.2017.02.011
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Abstract

Background  The ability of specific histopathological features to predict mortality or lung transplant in chronic hypersensitivity pneumonitis patients is unknown.

Methods  Patients with chronic hypersensitivity pneumonitis diagnosed by surgical lung biopsy were identified from an ongoing longitudinal cohort. The surgical lung biopsy slides were evaluated prospectively by an experienced thoracic pathologist using a standardized checklist to differentiate the major pathologic patterns and score the presence of specific histopathological features. Cox proportional hazard analysis was used to identify independent predictors of transplant-free survival, and Kaplan-Meier analysis was used to visualize outcomes.

Results  119 patients were identified. Patients with fibrotic non-specific interstitial pneumonia (f-NSIP) pattern, bronchiolocentric fibrosis (BF) pattern or usual interstitial pneumonia (UIP) pattern had significantly worse transplant-free survival than those with cellular NSIP (c-NSIP) pattern or peribronchiolar inflammation with poorly formed granulomas (PI-PFG) pattern. No survival difference among patients with f-NSIP pattern, BF pattern or UIP pattern was found. Fibroblastic foci were identified in a subset of biopsies from all pathological patterns. Peribronchiolar fibrosis was noted in all UIP cases. Independent predictors of time to death or transplant included the presence of fibroblast foci or dense collagen fibrosis.

Conclusions  Histopathologic patterns of c-NSIP and PI-PFG had a better transplant-free survival than UIP, f-NSIP and BF patterns. Presence of fibroblast foci or dense collagen fibrosis correlated with progression to death or lung transplantation. Identification of fibroblast foci on biopsies, regardless of the underlying histopathologic pattern, may be a clinically useful predictor of survival in HP patients.


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