The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype

Abstract

@ERSpublications ILDs with a progressive-fibrosing phenotype appear to be more common in older adults and are associated with a complex network of environmental and genetic factors. Further epidemiological studies are warranted to help identify these patients. http://ow.ly/SY6m30mWytM Cite this article as: Olson AL, Gifford AH, Inase N, et al. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev 2018; 27: 180077 [https://doi.ABSTRACT The availability of epidemiological data relating to interstitial lung diseases (ILDs) has increased over recent years, but information on the prevalence and incidence of ILDs of different aetiologies remains limited. Despite global distribution, the proportion of patients who develop a progressive phenotype across different ILDs is not well known. Disease behaviour is well documented in idiopathic pulmonary fibrosis but idiosyncratic in other ILDs that may present a progressive fibrosing phenotype. Possible reasons may include the heterogeneous nature of the aetiology, the complexity of diagnosis (and subsequent documentation of cases) and the methods employed to retrospectively analyse patient databases. This review presents a broad overview of the epidemiological data available for ILDs that may present a progressive-fibrosing phenotype, collectively and stratified according to clinical classification. We also note where further data are needed in comparison to the well-studied IPF indication.

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases

“…We believe that the healthcare utilisation and costs for other ILDs that may present a progressive fibrosing phenotype may be similar to those for IPF and therefore report findings known for IPF as a surrogate for detailed information regarding the collective diseases incorporated as PF-ILD [9,11]. The combined prevalence and duration of ILDs other than IPF with a progressive fibrosing phenotype are likely to equal or exceed those of IPF [12].…”

“…Despite this, up to 25% of patients remain unclassifiable following extensive investigation, which can be due to conflicting radiological or histopathological data or the unavailability of a lung biopsy [17,[21][22][23][24]. The epidemiology of ILDs that may present a progressive fibrosing phenotype is reviewed in [12]. Despite these shortcomings, the avoidance of invasive diagnostic procedures has been advocated where alternative diagnostic approaches are available, and this has the potential to reduce the use of healthcare resources.…”

The natural history of progressive fibrosing interstitial lung diseases

“…1). The proportion of patients with non-IPF ILDs who develop a progressive fibrosing phenotype is not known, but has been estimated by physicians who manage patients with non-IPF ILDs to be up to 40% [11,12]. In this review, we will describe the natural history of progressive fibrosing ILDs.…”

Enfermedades pulmonares intersticiales. Una perspectiva actual

“…La fibrosis pulmonar idiopática (FPI) es la patología más importante en este tipo de enfermedades por su prevalencia y pronóstico. Datos epidemiológicos recientes muestran que el número de enfermos va en aumento, aunque es posible que también exista mayor reconocimiento de la enfermedad y/o que se diagnostique en etapas más precoces 18,19 .…”