Chest
Original Research: Diffuse Lung DiseasePathologic Findings and Prognosis in a Large Prospective Cohort of Chronic Hypersensitivity Pneumonitis
Section snippets
Study Population
Patients with chronic HP and surgical lung biopsy samples were identified from a longitudinal cohort of patients followed at the University of California, San Francisco (UCSF) Interstitial Lung Disease Clinic from February 2003 to June 2013. The diagnosis of HP was based on consensus agreement by experts at a multidisciplinary conference after thorough review of all clinical, radiological, and pathologic data according to American Thoracic Society/European Respiratory Society guidelines.7, 12,
Clinical Characteristics
A total of 159 patients with a diagnosis of chronic HP who underwent surgical lung biopsy were identified from the UCSF Interstitial Lung Disease database. Of the 159 patients identified, 40 lacked pathologic scoring data in the database and were excluded, resulting in a study cohort of 119 patients. Baseline demographics are shown in Table 1. Antigen exposure categories included down, microbial, bird, soil/compost, and indoor hot tub (e-Table 1). Serum precipitant testing was not routinely
Discussion
This study of 119 prospectively enrolled patients with chronic HP and variable antigen exposure was designed to examine the frequencies of classifiable pathologic patterns and whether the histologic features were predictive of death or transplantation, or both. Our findings both extend and provide novel observations regarding the relationship between pathologic findings and clinical outcome. New observations include the finding that the pathologic patterns of c-NSIP and PI-PFG were associated
Conclusions
This study represents a prospective cohort of carefully scored lung biopsy samples from patients with chronic HP. In addition to major pathologic patterns, the clinical relevance of secondary histopathologic features was examined. Despite the finding that UIP and BF were not common in chronic HP, the presence of fibroblast foci could be identified in a subset of patients in all major pathologic patterns and predicted a poor outcome. Thus, the identification of fibroblast foci should be noted in
Acknowledgments
Author contributions: P. W. had full access to the data and takes responsibility for the integrity of the data and the accuracy of the data analysis. P. W. contributed to study design, data collection and analysis, statistical analysis, and writing, review, and approval of the final manuscript. K. D. J. and A. U. contributed to histopathologic slide review and assessment and review and approval of the final manuscript. B. M. E. and A. U. contributed to CT data collection and review in
References (22)
- et al.
Morphologic diversity of chronic pigeon breeder's disease: clinical features and survival
Respir Med
(2011) - et al.
The effect of pulmonary fibrosis on survival in patients with hypersensitivity pneumonitis
Am J Med
(2004) - et al.
Hypersensitivity pneumonitis: current concepts and future questions
J Allergy Clin Immunol
(2001) - et al.
Validation of a new dyspnea measure: the UCSD Shortness of Breath Questionnaire. University of California, San Diego
Chest
(1998) Fibroblastic foci: time to be counted?
Chest
(2006)- et al.
Hypersensitivity pneumonitis: a noninfectious granulomatosis
Semin Respir Infect
(1995) - et al.
Immunopathology, diagnosis, and management of hypersensitivity pneumonitis
Semin Respir Crit Care Med
(2012) - et al.
Hypersensitivity pneumonitis: a review and update of histologic findings
J Clin Pathol
(2013) - et al.
Chronic hypersensitivity pneumonitis
J Asthma Allergy
(2016) - et al.
Nonspecific interstitial pneumonitis: survival is influenced by the underlying cause
Eur Respir J
(2015)
Chronic bird fancier's lung: histopathologic and clinical correlation. An application of the 2002 ATS/ERS consensus classification of the idiopathic interstitial pneumonias
Thorax
Cited by (130)
Japanese clinical practice guide 2022 for hypersensitivity pneumonitis
2024, Respiratory InvestigationDistinct TNF-alpha and HLA polymorphisms associate with fibrotic and non-fibrotic subtypes of hypersensitivity pneumonitis
2023, PulmonologyCitation Excerpt :Whereas nonfibrotic or inflammatory subtype may have a benign course, fibrotic HP often present a progressive fibrotic phenotype compared to other fibrotic lung diseases.35 Definitely, similarities with IPF have been reported to be poor prognosis factors particularly the presence of usual interstitial pneumonia (UIP)-like pattern and absence of BAL lymphocytosis.50–54 Polymorphisms of the promoter regions of certain pro-inflammatory cytokines, such as IL-6 and IFN- γ may underlie the clinical heterogeneity of this disease.25,26
Real-world utility of a genomic classifier in establishing a diagnosis of newly identified interstitial lung disease
2023, Respiratory Medicine and ResearchUsual interstitial pneumonia as a stand-alone diagnostic entity: the case for a paradigm shift?
2023, The Lancet Respiratory Medicine
FUNDING/SUPPORT: This study was supported by departmental sources and the Nina Ireland Program for Lung Health.