title: A detailed evaluation of acute respiratory decline in patients with fibrotic lung disease: aetiology and outcomes.
abstract: OBJECTIVE
  A comprehensive diagnostic evaluation is recommended for all patients with fibrotic lung disease and acute respiratory decompensation. However, the effect on clinical outcomes of this evaluation remains unknown.
  
  
  METHODS
  We evaluated 27 consecutive patients with fibrotic lung disease who were hospitalized for an acute respiratory decline between June 2006 and April 2009. An interstitial lung disease expert assisted with the acute care of each patient. A retrospective review of the patient charts was performed to obtain demographic and clinical data, and to assess outcomes.
  
  
  RESULTS
  Using a strict definition of acute exacerbation (AE) of fibrotic lung disease derived from the IPF Network Pulmonary Perspective statement, 10 of the 27 patients were classified as definite AE and nine as suspected AE. In eight patients, infectious agents were identified as potential explanations for the respiratory decline. No patients with congestive heart failure or pulmonary embolism were identified. Overall survival to discharge was 37.0%. One-year survival was 14.8%. There were no differences in outcomes for patients with AE compared with those for whom potential infectious aetiologies were identified (log rank, P = 0.932). Patients with IPF showed a decreased rate of survival compared with patients with non-IPF fibrotic disease (1-year survival 0% vs 28.6%, log rank, P = 0.045).
  
  
  CONCLUSIONS
  In patients with fibrotic lung disease and an acute respiratory decline, a detailed diagnostic evaluation revealed a potential infectious aetiology in up to one-third of cases. However, there was no association between this finding and outcomes in these patients. One-year survival was dismal in patients who suffered an acute respiratory decompensation.
authors.affiliation: University of Colorado Denver, Division of Pulmonary Sciences and Critical Care Medicine, Aurora, Colorado 80045, USA. tristan.huie@ucdenver.edu
authors.forename: Tristan J
authors.initials: TJ
authors.lastname: Huie
authors.forename: Amy L
authors.initials: AL
authors.lastname: Olson
authors.forename: Gregory P
authors.initials: GP
authors.lastname: Cosgrove
authors.forename: William J
authors.initials: WJ
authors.lastname: Janssen
authors.forename: Abigail R
authors.initials: AR
authors.lastname: Lara
authors.forename: David A
authors.initials: DA
authors.lastname: Lynch
authors.forename: Steve D
authors.initials: SD
authors.lastname: Groshong
authors.forename: Marc
authors.initials: M
authors.lastname: Moss
authors.forename: Marvin I
authors.initials: MI
authors.lastname: Schwarz
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
authors.forename: Stephen K
authors.initials: SK
authors.lastname: Frankel
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000382:microbiology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000175:diagnosis* / Q000382:microbiology* / Q000401:mortality; D012131:Respiratory Insufficiency / Q000175:diagnosis / Q000382:microbiology / Q000401:mortality; D012189:Retrospective Studies; D016019:Survival Analysis