Predictors of survival in coexistent hypersensitivity pneumonitis with autoimmune features.
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.



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.



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.



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.
Department of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, 5841 S. Maryland Avenue, Chicago IL 60637, USA. Electronic address: deji@uchicago.edu.
Ayodeji
A
Adegunsoye
Department of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, 5841 S. Maryland Avenue, Chicago IL 60637, USA.
Justin M
JM
Oldham
Department of Medicine, The University of Chicago, 5841 S. Maryland Avenue, Chicago IL 60637, USA.
Carley
C
Demchuk
Department of Radiology, The University of Chicago, 5841 S. Maryland Avenue, Chicago IL 60637, USA.
Steven
S
Montner
Department of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, 5841 S. Maryland Avenue, Chicago IL 60637, USA.
Rekha
R
Vij
Department of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, 5841 S. Maryland Avenue, Chicago IL 60637, USA.
Mary E
ME
Strek
D000368:Aged; D000542:Alveolitis, Extrinsic Allergic / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000276:immunology* / Q000473:pathology; D000941:Antigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology*; D015551:Autoimmunity; D003240:Connective Tissue Diseases / Q000150:complications* / Q000175:diagnosis / Q000276:immunology*; D003937:Diagnosis, Differential; D004781:Environmental Exposure / Q000009:adverse effects; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D015995:Prevalence; D011658:Pulmonary Fibrosis / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality; D012189:Retrospective Studies; D016019:Survival Analysis
D000941:Antigens; D007166:Immunosuppressive Agents
Autoimmunity; Connective tissue disease; Hypersensitivity pneumonitis; Immunosuppressive therapy; Interstitial lung disease; Pulmonary fibrosis