Faculty Collaboration Database

Predictors of survival in coexistent hypersensitivity pneumonitis with autoimmune features. Respir Med 2016 May;114:53-60

Date

04/26/2016

Pubmed ID

27109811

Pubmed Central ID

PMC4845760

DOI

10.1016/j.rmed.2016.03.012

Scopus ID

2-s2.0-84961707774 (requires institutional sign-in at Scopus site)   47 Citations

Abstract

BACKGROUND: Hypersensitivity pneumonitis (HP), an immune-mediated inflammatory interstitial lung disease (ILD), can result from exposure to several well-recognized antigens. Despite antigen avoidance, progressive pulmonary fibrosis and death can occur, suggesting that additional factors may contribute to disease activity. We hypothesized that the presence of autoimmunity might impact clinical course in patients with HP. In this study, we examined an HP cohort to identify those with HP and autoimmune features (HPAF), and determine its prevalence and outcomes.

METHODS: The University of Chicago ILD registry was screened to identify patients with HP. Patients were characterized as HPAF if they had an autoimmune disease or features of autoimmunity, defined as the presence of specific connective tissue disease (CTD) symptoms and serologies. Demographics, clinical characteristics, and outcomes were compared between groups. Survival analysis was performed using Cox regression to identify predictors of transplant-free survival in this cohort.

RESULTS: One hundred twenty patients with chronic, fibrotic HP were identified. Of these, 18/120 (15%) were characterized as HPAF. Compared to those without evidence of autoimmunity, patients with HPAF had a higher proportion of females (54% vs. 83%, respectively; p = 0.02) but were otherwise similar with regard to clinical characteristics. The presence of autoimmunity was an independent predictor of increased mortality (HR 4.45; 95% CI 1.43-13.88; p = 0.01) after multivariable adjustment.

CONCLUSIONS: Fifteen percent of patients with chronic, fibrotic HP displayed evidence of a concurrent defined autoimmune disease or autoimmune features suggestive of CTD. The presence of autoimmunity in patients with chronic, fibrotic HP may portend a poorer prognosis. Future studies are needed to validate these findings and determine the impact of immunosuppressive treatment.

Author List

Adegunsoye A, Oldham JM, Demchuk C, Montner S, Vij R, Strek ME

Author

Carley Demchuk MD Assistant Professor in the Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Aged
Alveolitis, Extrinsic Allergic
Antigens
Autoimmune Diseases
Autoimmunity
Connective Tissue Diseases
Diagnosis, Differential
Environmental Exposure
Female
Humans
Immunosuppressive Agents
Lung Diseases, Interstitial
Male
Middle Aged
Predictive Value of Tests
Prevalence
Pulmonary Fibrosis
Retrospective Studies
Survival Analysis