Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review

Abstract

As idiopathic pulmonary fibrosis emerges as an important public health problem, there is a need to coordinate data on incidence and mortality globally. This study aims to systematically assess all available studies to investigate the global burden of disease.Medline and Embase databases were searched systematically for all population-based studies of incidence or mortality of idiopathic pulmonary fibrosis. Clinical case series and prevalence studies were excluded. The search was supplemented using the Google search engine, hand-searching of references and conference abstracts. Data were extracted independently by two authors using a pre-specified proforma, with assessment of methodological quality.34 studies were identified, providing data from 21 countries from 1968-2012. 28 studies reported incidence data and eight reported mortality data. In studies from the year 2000 onwards, we estimated a conservative incidence range of 3-9 cases per 100 000 per year for Europe and North America. Incidence was lower in East Asia and South America. The majority of studies showed an increase in incidence over time.The incidence of idiopathic pulmonary fibrosis is increasing worldwide and rates are coming together across countries. Current data suggest incidence is similar to that of conditions such as stomach, liver, testicular and cervical cancers. @ERSpublications Incidence of IPF varies worldwide but seems to be increasing to rates around 3-9 per 100 000 per year http://ow.ly/KSeMZ

Influence of Idiopathic Pulmonary Fibrosis Progression on Healthcare Resource Use

“…There were 35 (0.9%) observed investigator-reported acute exacerbations. 3 The value reflects the FVC%pred at the beginning of any of the available 3-month intervals (3774) 3 All exacerbation events were considered for the period from baseline until the day of the last FVC measure in the trial period for each patient (circa 12 months).…”

“…More than 5000 new cases occur annually in the UK alone [2], with an incidence of three to nine cases per 100,000 per year in Europe and North America [3]. The disease rarely occurs in people aged \ 45 years.…”

Die idiopathische Lungenfibrose

“…Daten aus Groß-britannien lassen eine Inzidenz von 8,6 Patienten/ 100 000 Einwohner in Deutschland vermuten [1]. Die IPF gehört damit noch zu den seltenen Lungenerkrankungen und Medikamente für die IPF tragen einen "orphan drug" Status [2].…”

Idiopathic pulmonary fibrosis: another step in understanding the burden of this disease

“…These later studies boasted far greater numbers, though with some concern about the validity of the cases, the reliability of clinical coding and generalisability to the wider population. A recent systematic review estimated the incidence of IPF to be 3-9 cases per 100 000 in Europe and North America, although this included a heterogenous mix of studies with different case definitions and populations, and several less reliable estimates had to be excluded [2]. Therefore, identifying the true incidence of IPF remains a challenge [11].…”

“…The past 25 years have seen a steady increase in the number of studies examining the incidence of idiopathic pulmonary fibrosis (IPF) worldwide [1,2]. In general, early studies tended to involve clinicians collating cases from their local area [3,4] or asking interested colleagues to contribute to registries [5,6], whereas later studies have made use of large databases collected for clinical care or administrative reasons [7][8][9][10].…”