Association between FVC and mortality or survival in idiopathic pulmonary fibrosis: a systematic literature review

Abstract

Aims and objectives: We reviewed the influence of FVC on mortality and survival in patients with IPF managed in real-world settings.

Methods: A systematic literature review was performed to determine the association of FVC and change in FVC over time with mortality and survival in patients with IPF aged ≥18 years. A search was conducted in Embase and Medline on December 1, 2020, from 2009 onwards. Conference abstracts were captured through Embase or manual searches. All identified publications were screened by two reviewers for inclusion.

Results: Of 1696 publications screened, 88 were included in the review (55 journal articles; 39 abstracts). The majority of studies were performed in Japan (n = 20) and the US (n = 18). The mean age was 53–75 years; patients were predominately male with a median time from symptom onset of 5 to 24 months. Baseline FVC and %predicted change was significantly associated with mortality in 75% (n/N, 6/8) and 85% (17/20) of studies and with survival in 67% (4/6) and 100% (13/13) of studies. Although FVC cut-off points varied across studies, an FVC% predicted less than 80% at baseline was generally associated with higher mortality. A significant association between FVC decline and increased mortality or lower survival was reported in 82% (14/17) of studies. A decrease in FVC ≥10% from baseline to 12 months was associated with survival in 3 of 3 studies and mortality in 2 of 3 studies.

Conclusions: In the real-world settings, lower baseline FVC and FVC decline over time are consistently associated with increased mortality and decreased survival, although FVC cut-off points varied across studies.

Funding: Galapagos