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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 53-60 |
| Number of pages | 8 |
| Journal | Respiratory Medicine |
| Volume | 114 |
| DOIs | |
| State | Published - May 1 2016 |
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Keywords
- Autoimmunity
- Connective tissue disease
- Hypersensitivity pneumonitis
- Immunosuppressive therapy
- Interstitial lung disease
- Pulmonary fibrosis
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
Cite this
Predictors of survival in coexistent hypersensitivity pneumonitis with autoimmune features. / Adegunsoye, Ayodeji; Oldham, Justin; Demchuk, Carley; Montner, Steven; Vij, Rekha; Strek, Mary E.
In: Respiratory Medicine, Vol. 114, 01.05.2016, p. 53-60.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Predictors of survival in coexistent hypersensitivity pneumonitis with autoimmune features
AU - Adegunsoye, Ayodeji
AU - Oldham, Justin
AU - Demchuk, Carley
AU - Montner, Steven
AU - Vij, Rekha
AU - Strek, Mary E.
PY - 2016/5/1
Y1 - 2016/5/1
N2 - 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.
AB - 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.
KW - Autoimmunity
KW - Connective tissue disease
KW - Hypersensitivity pneumonitis
KW - Immunosuppressive therapy
KW - Interstitial lung disease
KW - Pulmonary fibrosis
UR - http://www.scopus.com/inward/record.url?scp=84961707774&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84961707774&partnerID=8YFLogxK
U2 - 10.1016/j.rmed.2016.03.012
DO - 10.1016/j.rmed.2016.03.012
M3 - Article
C2 - 27109811
AN - SCOPUS:84961707774
VL - 114
SP - 53
EP - 60
JO - Respiratory Medicine
JF - Respiratory Medicine
SN - 0954-6111
ER -