Journal Article

Delayed Access and Survival in Idiopathic Pulmonary Fibrosis: A Cohort Study

Lamas, Daniela J., Bagiella, Emilia, Kawut, Steven M., Arcasoy, Selim M., Lederer, David J., Philip, Nisha

2011

Overview

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.

• Publisher: Oxford University Press,American Thoracic Society
• Subject: Adult / Aged / Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy / Biological and medical sciences / Childrens health insurance programs / Cohort analysis / Delayed Diagnosis - mortality / Dyspnea / Educational attainment / Female / Health care policy / Health insurance / Health Services Accessibility / Healthcare Disparities / Humans / Idiopathic Pulmonary Fibrosis - diagnosis / Idiopathic Pulmonary Fibrosis - mortality / Insurance coverage / Intensive care medicine / Lung diseases / Lung transplants / Male / Medical records / Medical sciences / Middle Aged / New York City - epidemiology / Pneumology / Prospective Studies / Pulmonary fibrosis / Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases / Questionnaires / Referral and Consultation / Risk Factors / Society / Survival Rate