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Am J Respir Crit Care Med. 2011 Oct 1;184(7):842-7. doi: 10.1164/rccm.201104-0668OC.

Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study.

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  • 1Department of Medicine, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA.

Abstract

RATIONALE:

Idiopathic pulmonary fibrosis is often initially misdiagnosed. Delays in accessing subspecialty care could lead to worse outcomes among those with idiopathic pulmonary fibrosis.

OBJECTIVES:

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.

MEASUREMENTS AND MAIN 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.

PMID:
21719755
[PubMed - indexed for MEDLINE]
PMCID:
PMC3208648
Free PMC Article
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