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Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis


Zheng, Q and Cox, IA and Campbell, JA and Xia, Q and Otahal, P and de Graaff, B and Corte, TJ and Teoh, AKY and Walters, EH and Palmer, AJ, Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis, ERJ Open Research, 8, (1) Article 00591-2021. ISSN 2312-0541 (2022) [Refereed Article]

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ŠThe authors 2022. This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0 International (CC BY-NC 4.0)

DOI: doi:10.1183/23120541.00591-2021


Background:There are substantial advances in diagnosis and treatment for idiopathic pulmonary fibrosis (IPF), but without much evidence available on recent mortality and survival trends.

Methods: A narrative synthesis approach was used to investigate the mortality trends, then meta-analyses for survival trends were carried out based on various time periods. Results: Six studies reported the mortality data for IPF in 22 countries, and 62 studies (covering 63,307 patients from 20 countries) reported survival data for IPF. Age-standardised mortality for IPF varied from approximately 0.5 to 12 per 100,000 population per year after year 2000. There were increased mortality trends for IPF in Australia, Brazil, Belgium, Canada, Czech Republic, Finland, France, Germany, Hungary, Italy, Lithuania, Netherlands, Poland, Portugal, Spain, Sweden, and UK, while Austria, Croatia, Denmark, Romania, and US showed decreased mortality trends. The overall 3-year and 5-year cumulative survival rates (CSRs) were 61.8% (95% CI, 58.7-64.9; I2=97.1%) and 45.6% (95% CI, 41.5-49.7; I2=97.7%), respectively. Prior to 2010, the pooled 3-year CSRs was 59.9% (95% CI, 55.8-64.1; I2>95.8%), then not significantly (P=0.067) increased to 66.2% (95% CI, 62.9-69.5; I2=92.6%) in the 2010s decade. After excluding three studies in which no patients received antifibrotics after year 2010, the pooled 3-year CSRs significantly (P=0.039) increased to 67.4% (95% CI, 63.9- 70.9; I2=93.1%) in the 2010s decade.

Discussion: IPF is a diagnosis associated with high mortality. There was no observed increasing survival trend for patients with IPF before year 2010, with then a switch to an improvement, which is probably multifactorial.

Item Details

Item Type:Refereed Article
Keywords:idiopathic pulmonary fibrosis, respiratory disease, mortality, survival, epidemiology, burden of disease
Research Division:Biomedical and Clinical Sciences
Research Group:Cardiovascular medicine and haematology
Research Field:Respiratory diseases
Objective Division:Health
Objective Group:Public health (excl. specific population health)
Objective Field:Disease distribution and transmission (incl. surveillance and response)
UTAS Author:Zheng, Q (Mr Qiang Zheng)
UTAS Author:Cox, IA (Dr Ingrid Cox)
UTAS Author:Campbell, JA (Dr Julie Campbell)
UTAS Author:Xia, Q (Miss Qing Xia)
UTAS Author:Otahal, P (Mr Petr Otahal)
UTAS Author:de Graaff, B (Dr Barbara de Graaff)
UTAS Author:Walters, EH (Professor Haydn Walters)
UTAS Author:Palmer, AJ (Professor Andrew Palmer)
ID Code:148416
Year Published:2022
Web of Science® Times Cited:2
Deposited By:Menzies Institute for Medical Research
Deposited On:2022-01-10
Last Modified:2022-12-06
Downloads:19 View Download Statistics

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