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. 2016 Feb 3;11(2):e0147072.
doi: 10.1371/journal.pone.0147072. eCollection 2016.

Epidemiology of Idiopathic Pulmonary Fibrosis in Northern Italy

Sergio Harari  1 , Fabiana Madotto  2 , Antonella Caminati  1 , Sara Conti  2 , Giancarlo Cesana  2
Affiliations

Epidemiology of Idiopathic Pulmonary Fibrosis in Northern Italy

Sergio Harari et al. PLoS One. .

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic interstitial pneumonia. Despite its clinical relevance, few studies have examined the epidemiology of IPF and temporal variation in disease incidence and prevalence. Aim of the study was to investigate the prevalence, incidence and trends of IPF in Lombardy, a region with nearly 10 million inhabitants, during 2005-2010.

Methods: For the identification of IPF patients, we used healthcare administrative databases of Lombardy Healthcare System and adopted three algorithms: generic, broad and narrow case definition (GCD, BCD, NCD). IPF cases were identified according to diagnoses reported in inpatient and outpatient claims occurred during 2000-2010. We estimated age- and sex-adjusted annual prevalence and incidence rates from 2005 to 2010, thus allowing for a 5-year washout period.

Results: The mean annual incidence rate was estimated at 2.3 and 5.3 per 100,000 person-years using NCD and GCD, respectively. IPF incidence was higher among males, and increased with age. Trend remained stable over the years. The estimated annual prevalence rate was 35.5, 22.4, and 12.6 per 100,000 person-years using GCD, BCD and NCD, respectively, and increased with age. Moreover, we observed a positive trend over the years. Using BCD and NCD, prevalence was higher among males.

Conclusions: The results of this study, which is one of the largest population-based survey ever conducted according to strict criteria, indicated that prevalence of IPF increased across the years while incidence remained stable, thus suggesting that survival with IPF has improved.

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Conflict of interest statement

Competing Interests: Outside the submitted work, Dr. Harari reports personal fees from InterMune s.r.l, Roche, Boehringer Ingelheim and Dr. Caminati from InterMune s.r.l., Roche and Boehringer Ingelheim Italia s.p.a. The Research Center on Public Health received a grant from InterMune s.r.l. for taking part in the project that originated this publication, but no personal fees were received by Dr. Cesana, Madotto and Conti.

Figures

Fig 1
Fig 1. Trend in age-sex standardized annual prevalence rates (per 100,000 person-years) of IPF in Lombardy during 2005–2010, by case definition.
(A) General case definition. (B) Broad case definition. (C) Narrow case definition. Grey bands indicate 95% confidence intervals.
Fig 2
Fig 2. Trend in age-sex standardized annual incidence rates (per 100,000 person-years) of IPF in Lombardy during 2005–2010 by case definition.
(A) General case definition. (B) Broad case definition. (C) Narrow case definition. Grey bands indicate 95% confidence intervals.
See this image and copyright information in PMC

References

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