Incidence and Prevalence of Idiopathic Pulmonary Fibrosis

University of Washington Medical Center, Seattle, WA 98195-6522, USA.
American Journal of Respiratory and Critical Care Medicine (Impact Factor: 13). 11/2006; 174(7):810-6. DOI: 10.1164/rccm.200602-163OC
Source: PubMed


Idiopathic pulmonary fibrosis is a chronic interstitial lung disease of unknown etiology; its epidemiology in the United States has not been well characterized.
To estimate the annual incidence and prevalence of idiopathic pulmonary fibrosis in the United States.
Retrospective cohort design utilizing a large health care claims database spanning the period January 1996 through December 2000.
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 to 34 yr to 227.2 per 100,000 among those 75 yr or older; 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 United States' 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).
Our results suggest that idiopathic pulmonary fibrosis is probably more common in the United States than previously reported.

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    • "Moreover, thanks to the universal health care coverage and to the DENALI data warehouse, which traces a complete medical history of each resident in Lombardy by merging data of different datasets belonging to the same individual, we could investigate the prevalence and incidence of IPF in an unselected population, without restrictions related to age [11,13,31], adherence to some health plan (e.g. Medicare) [11,28], or voluntary recruitment [17,32]. In conclusion, our results on IPF prevalence and incidence are in line with those reported in other epidemiological studies conducted in Italy and Europe, and incidence and prevalence trends are in agreement with those reported in European and American studies. "
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    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.
    Full-text · Article · Feb 2016 · PLoS ONE
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    • "BioMed Research International but is present in approximately 0.2% of those older than 75 [3] [4]. The incidence of IPF, estimated at 3–9 cases per 100,000 per year in Europe and North America, is increasing worldwide [5], and IPF is also likely to account for much of the increased ILD-related mortality reported worldwide between 1990 and 2013 [6]. "
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    ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is a devastating condition with a 5-year survival of approximately 20%. The disease primarily occurs in elderly patients. IPF is a highly heterogeneous disorder with a clinical course that varies from prolonged periods of stability to episodes of rapid deterioration. In the last decade, improved understanding of disease mechanisms along with a more precise disease definition has allowed the design and completion of a number of high-quality clinical trials. Yet, until recently, IPF was essentially an untreatable disease. Finally, pirfenidone and nintedanib, two compounds with antifibrotic properties, have consistently proven effective in reducing functional decline and disease progression in IPF. This is a major breakthrough for patients and physicians alike, but there is still a long way to go. In fact, neither pirfenidone nor nintedanib is a cure for IPF, and most patients continue to progress despite treatment. As such, comprehensive care of patients with IPF, including management of comorbidities/complications and physical debility and timely referral for palliative care or, in a small number of highly selected patients, lung transplantation, remains essential. Several agents with high potential are currently being tested and many more are ready to be evaluated in clinical trials.
    Full-text · Article · Dec 2015
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    • "occurs in middle-aged and elderly adults, and its incidence increases with age; most IPF patients are older than 60 years at the time of diagnosis[1,585960. However, the usual interstitial pneumonia (UIP) pattern of lung fibrosis, which is the histologic appearance of the fibrotic pattern of IPF, also occurs in a small number of young people. "
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    ABSTRACT: Individual alveolar epithelial cells (AECs) collaboratively form a tight barrier between atmosphere and fluid-filled tissue to enable normal gas exchange. The tight junctions of AECs provide intercellular sealing and are integral to the maintenance of the AEC barrier integrity. Disruption and failure of reconstitution of AEC barrier result in catastrophic consequences, leading to alveolar flooding and subsequent devastating fibrotic scarring. Recent evidences reveal that many of the fibrotic lung diseases involve AECs both as a frequent target of injury and as a driver of ongoing pathological processes. Aberrantly activated AECs express most of the growth factors and chemokines responsible for the proliferation, migration, and activation of fibroblasts. Current evidences suggest that AECs may acquire overdrive activation in the initial step of fibrosis by several mechanisms, including abnormal recapitulation of the developmental pathway, defects of the molecules essential for epithelial integrity, and acceleration of aging-related properties. Among these initial triggering events, epithelial Pten, a multiple phosphatase that negatively regulates the PI3K/Akt pathway and is crucial for lung development, is essential for the prevention of alveolar flooding and lung fibrosis through the regulation of AEC barrier integrity after injury. Reestablishment of AEC barrier integrity also involves the deployment of specialized stem/progenitor cells.
    Full-text · Article · Oct 2015
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