title: Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis.
abstract: BACKGROUND
  The cornerstone of hypersensitivity pneumonitis (HP) management is having patients avoid the inciting antigen (IA). Often, despite an exhaustive search, an IA cannot be found. The objective of this study was to examine whether identifying the IA impacts survival in patients with chronic HP.
  
  
  METHODS
  We used the Kaplan-Meier method to display, and the log-rank test to compare, survival curves of patients with well-characterized chronic HP stratified on identification of an IA exposure. A Cox proportional hazards (PH) model was used to identify independent predictors in time-to-death analysis.
  
  
  RESULTS
  Of 142 patients, 67 (47%) had an identified IA, and 75 (53%) had an unidentified IA. Compared with survivors, patients who died (n = 80, 56%) were older, more likely to have smoked, had lower total lung capacity % predicted and FVC % predicted, had higher severity of dyspnea, were more likely to have pulmonary fibrosis, and were less likely to have an identifiable IA. In a Cox PH model, the inability to identify an IA (hazard ratio [HR], 1.76; 95% CI, 1.01-3.07), older age (HR, 1.04; 95% CI, 1.01-1.07), the presences of pulmonary fibrosis (HR, 2.43; 95% CI, 1.36-4.35), a lower FVC% (HR, 1.36; 95% CI, 1.10-1.68), and a history of smoking (HR, 2.01; 95% C1, 1.15-3.50) were independent predictors of shorter survival. After adjusting for mean age, presence of fibrosis, mean FVC%, mean diffusing capacity of the lung for carbon monoxide (%), and history of smoking, survival was longer for patients with an identified IA exposure than those with an unidentified IA exposure (median, 8.75 years vs 4.88 years; P = .047).
  
  
  CONCLUSIONS
  Among patients with chronic HP, when adjusting for a number of potentially influential predictors, including the presence of fibrosis, the inability to identify an IA was independently associated with shortened survival.
authors.affiliation: Interstitial Lung Disease Program and Autoimmune Lung Center, National Jewish Health, Denver, CO. Electronic address: Fernandezevans@njhealth.org.
authors.forename: Evans R
authors.initials: ER
authors.lastname: Fernández Pérez
authors.affiliation: Interstitial Lung Disease Program and Autoimmune Lung Center, National Jewish Health, Denver, CO.
authors.forename: Jeffrey J
authors.initials: JJ
authors.lastname: Swigris
authors.affiliation: Division of Pulmonary and Critical Care Medicine, and the Department of Biostatistics and Bioinformatics, National Jewish Health, Denver, CO.
authors.forename: Anna V
authors.initials: AV
authors.lastname: Forssén
authors.affiliation: Calgary Medical Center, Calgary, AB, Canada.
authors.forename: Olga
authors.initials: O
authors.lastname: Tourin
authors.affiliation: Interstitial Lung Disease Program and Autoimmune Lung Center, National Jewish Health, Denver, CO.
authors.forename: Joshua J
authors.initials: JJ
authors.lastname: Solomon
authors.affiliation: Interstitial Lung Disease Program and Autoimmune Lung Center, National Jewish Health, Denver, CO.
authors.forename: Tristan J
authors.initials: TJ
authors.lastname: Huie
authors.affiliation: Interstitial Lung Disease Program and Autoimmune Lung Center, National Jewish Health, Denver, CO.
authors.forename: Amy L
authors.initials: AL
authors.lastname: Olson
authors.affiliation: Interstitial Lung Disease Program and Autoimmune Lung Center, National Jewish Health, Denver, CO.
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
mesh_terms: D000416:Alberta / Q000453:epidemiology; D000542:Alveolitis, Extrinsic Allergic / Q000276:immunology* / Q000401:mortality; D002908:Chronic Disease; D005260:Female; D000949:Histocompatibility Antigens Class II / Q000032:analysis* / Q000276:immunology; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013997:Time Factors
chemical_list: D000949:Histocompatibility Antigens Class II