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Review
. 2022 Mar 14;8(1):00591-2021.
doi: 10.1183/23120541.00591-2021. eCollection 2022 Jan.

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

Qiang Zheng  1   2   3 , Ingrid A Cox  1   2 , Julie A Campbell  1 , Qing Xia  1 , Petr Otahal  1 , Barbara de Graaff  1 , Tamera J Corte  2   4   5 , Alan K Y Teoh  2   4   5 , E Haydn Walters  6   7   8 , Andrew J Palmer  1   2   7   8
Affiliations
Review

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

Qiang Zheng et al. ERJ Open Res. .

Abstract

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 ∼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, the Netherlands, Poland, Portugal, Spain, Sweden and UK, while Austria, Croatia, Denmark, Romania and the USA 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 CSR 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.

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Conflict of interest statement

Conflict of interest: T.J. Corte reports grants, personal fees and nonfinancial support from Boehringer Ingelheim and Hoffman La Rochel grants and personal fees from Bristol Myers Squibb; grants from Avalyn Pharma and Biogen; and personal fees from Promedior, outside the submitted work. A.K.Y. Teoh reports conference fees from Boehringer Ingelheim and speaker fees from Roche outside the submitted work. The other co-authors declare no competing interests.

Figures

FIGURE 1
FIGURE 1
Flow diagram of search progress, informed by PRISMA guidelines.
FIGURE 2
FIGURE 2
a) and c) Subgroup analyses for survival rates by various time periods; b) and d) after exclusion of three studies in which no patients received antifibrotics after year 2010. I2>50% represents high between-studies heterogeneity. IPF: idiopathic pulmonary fibrosis; n: number of included studies.
FIGURE 3
FIGURE 3
Subgroup analyses for cumulative survival rates (CSRs) by various pharmaceutical regimens. a) 3-year CSRs; b) 5-year CSRs. a) Test for difference between subgroups = 0.032; b) test for difference between subgroups = 0.084.
FIGURE 4
FIGURE 4
Association between mean age at diagnosis and median year studied between 1960 and 2020 by using univariate meta-regression. Each size of the bubble depends on the weights in the random-effects models. Orange circles show studies removed for sensitivity analysis with extreme data points or before year 1980. Coef.: coefficient.
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References

    1. Lederer DJ, Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med 2018; 378: 1811–1823. doi: 10.1056/NEJMra1705751 - DOI - PubMed
    1. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. The Lancet 2017; 389: 1941–1952. doi: 10.1016/S0140-6736(17)30866-8 - DOI - PubMed
    1. Hutchinson J, Fogarty A, Hubbard R, et al. Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. Eur Respir J 2015; 46: 795–806. doi: 10.1183/09031936.00185114 - DOI - PubMed
    1. Levin KA. Study design VI – ecological studies. Evid Based Dent 2006; 7: 108. doi: 10.1038/sj.ebd.6400454 - DOI - PubMed
    1. Khor YH, Ng Y, Barnes H, et al. Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: a systematic review. Eur Respir Rev 2020; 29: 190158. doi: 10.1183/16000617.0158-2019 - DOI - PMC - PubMed

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