title: Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study.
abstract: BACKGROUND
  Idiopathic pulmonary fibrosis is often initially misdiagnosed. Delays in accessing subspecialty care could lead to worse outcomes among those with idiopathic pulmonary fibrosis.
  
  
  OBJECTIVE
  To examine the association between delayed access to subspecialty care and survival time in idiopathic pulmonary fibrosis.
  
  
  METHODS
  We performed a prospective cohort study of 129 adults who met American Thoracic Society criteria for idiopathic pulmonary fibrosis evaluated at a tertiary care center. Delay was defined as the time from the onset of dyspnea to the date of initial evaluation at a tertiary care center. We used competing risk survival methods to examine survival time and time to transplantation.
  
  
  RESULTS
  The mean age was 63 years and 76% were men. The median delay was 2.2 years (interquartile range 1.0–3.8 yr), and the median follow-up time was 1.1 years. Age and lung function at the time of evaluation did not vary by delay. A longer delay was associated with an increased risk of death independent of age, sex, forced vital capacity, third-party payer, and educational attainment (adjusted hazard ratio per doubling of delay was 1.3, 95% confidence interval 1.03 to 1.6). Longer delay was not associated with a lower likelihood of undergoing lung transplantation.
  
  
  CONCLUSIONS
  Delayed access to a tertiary care center is associated with a higher mortality rate in idiopathic pulmonary fibrosis independent of disease severity. Early referral to a specialty center should be considered for those with known or suspected interstitial lung disease.
authors: Department of Medicine, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA.
authors: Daniela J
authors: DJ
authors: Lamas
authors: Steven M
authors: SM
authors: Kawut
authors: Emilia
authors: E
authors: Bagiella
authors: Nisha
authors: N
authors: Philip
authors: Selim M
authors: SM
authors: Arcasoy
authors: David J
authors: DJ
authors: Lederer
mesh_terms: D000328:Adult; D000368:Aged; D057210:Delayed Diagnosis / Q000401:mortality*; D005260:Female; D006297:Health Services Accessibility; D054625:Healthcare Disparities; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D009519:New York City / Q000453:epidemiology; D011446:Prospective Studies; D012017:Referral and Consultation; D012307:Risk Factors; D015996:Survival Rate