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 pp. 1-43. ISSN 2312-0541 (In Press) [Refereed Article]
Background: There are substantial advances in diagnosis and treatment for idiopathic
pulmonary fibrosis (IPF), but without much evidence available on recent mortality and survival
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.