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Published ahead of print on June 29, 2006, doi:10.1164/rccm.200602-163OC

Am. J. Respir. Crit. Care Med., Volume 174, Number 7, October 2006, 810-816

A more recent version of this article appeared on October 1, 2006
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Submitted on February 3, 2006
Accepted on June 28, 2006

Incidence and Prevalence of Idiopathic Pulmonary Fibrosis

Ganesh Raghu1*, Derek Weycker2, John Edelsberg2, Williamson Z Bradford3, and Gerry Oster2

1 University of Washington Medical Center, Seattle, WA, USA, 2 Policy Analysis Inc. (PAI), Brookline, MA, USA, 3 InterMune Pharmaceuticals, Brisbane, CA, USA

* To whom correspondence should be addressed. E-mail: graghu{at}u.washington.edu.

Rationale. Idiopathic pulmonary fibrosis is a chronic interstitial lung disease of unknown etiology; its epidemiology in the United States has not been well characterized. Objective. To estimate the annual incidence and prevalence of idiopathic pulmonary fibrosis in the United States. Methods. Retrospective cohort design utilizing a large healthcare claims database spanning the period January 1996 through December 2000. Measurements and Main Results. Persons with idiopathic pulmonary fibrosis were identified based on diagnosis and procedure codes. Using broad case-finding criteria, prevalence was estimated to range from 4.0 per 100,000 persons aged 18-34 years to 227.2 per 100,000 among those ≥ 75 years of age; annual incidence was estimated to range from 1.2 to 76.4 per 100,000. Using narrow case-finding criteria, prevalence ranged from 0.8 to 64.7 per 100,000 persons; comparable figures for incidence were 0.4 to 27.1 per 100,000 persons. Extrapolating these rates to the overall US population, prevalence was estimated to be 42.7 per 100,000 (incidence, 16.3 per 100,000) using broad criteria; with narrow criteria, prevalence was estimated to be 14.0 per 100,000 (incidence, 6.8 per 100,000). Conclusions. Our results suggest that idiopathic pulmonary fibrosis is probably more common in the United States than previously reported.
Key words: lung diseases, interstitial; epidemiology; retrospective studies




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