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

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 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.

Deficiency in the zinc transporter ZIP8 impairs epithelia renewal and enhances lung fibrosis

“…However, in idiopathic pulmonary fibrosis (IPF), the normal honeycomb structure of alveoli is replaced by fibrotic tissue, resulting in reduced gas exchange in early stages of the disease, which ultimately leads to death from pulmonary failure. IPF is essentially an intractable disease, although some headway has been made with antifibrotics: 5-year cumulative survival rates are 51.4% for people taking antifibrotics versus 43.9% for people not taking these drugs ( 1 ), and a 2.5%–4.3% reduction in the decline of forced viral capacity was reported across various antifibrotics in a recent meta-analysis ( 2 ). Even with current therapeutics, the prognosis for IPF patients is worse than for most cancers, including lung cancer.…”

Developing a self-management package for pulmonary fibrosis: an international Delphi study

“…A recent systematic review reported significantly better survival among those taking antifibrotics [6].…”

“…A recent systematic review reported significantly better survival among those taking antifibrotics [ 6 ]. With longer survival comes the need to more effectively manage the disease.…”

Present and future perspectives in early diagnosis and monitoring for progressive fibrosing interstitial lung diseases

“…Within the complex landscape of interstitial lung diseases (ILDs), a widely studied disease and a major new concept have emerged with the publication of the updated 2022 ATS/ERS/JRS/ALAT clinical practice guideline: progressive pulmonary fibrosis (PPF). ( 1 ) While idiopathic pulmonary fibrosis (IPF) is a diagnosis of exclusion, with an unknown etiology and a grim prognosis rivaling most cancers, ( 2 ) PPF includes a multitude of ILDs, of both known and unknown origin, that share a progressive disease behavior.…”