Diagnostic delay in IPF impacts progression-free survival, quality of life and hospitalisation rates

Nils Hoyer, Thomas Skovhus Prior, Elisabeth Bendstrup, Saher Burhan Shaker, Nils Hoyer, Thomas Skovhus Prior, Elisabeth Bendstrup, Saher Burhan Shaker

Abstract

Background: The diagnosis of idiopathic pulmonary fibrosis (IPF) is often delayed up to several years. The objective of this study was to assess the impact of the diagnostic delay on progression-free survival, quality of life and hospitalisation rates.

Methods: A total of 264 incident patients with IPF were included immediately after their diagnosis and followed for up to 5 years, with regular collection of clinical data, quality-of-life questionnaires and assessment of disease progression. Hospitalisation data were extracted from electronic patient records. Analyses were performed on the entire cohort and strata according to forced vital capacity (FVC) at diagnosis.

Results: A long diagnostic delay (>1 year) was associated with worse progression-free survival compared with a short diagnostic delay (<1 year) (HR: 1.70, 95% CI: 1.18 to 2.46, p=0.004) especially in patients with mild disease at the time of diagnosis (FVC>80% predicted). Mean total scores of the St. George's respiratory questionnaire (SGRQ), a derived IPF-specific version of the SGRQ and the chronic obstructive pulmonary disease assessment test (CAT) were consistently higher in patients with long diagnostic delays, indicating worse quality of life. Mean hospitalisation rates were higher during the first year after diagnosis (Incidence rate ratio [IRR]: 3.28, 95% CI: 1.35 to 8.55, p=0.01) and during the entire follow-up (IRR: 1.74, 95% CI: 1.01 to 3.02, p=0.04).

Conclusion: A diagnostic delay of more than 1 year negatively impacts progression-free survival, quality of life and hospitalisation rates in patients with IPF. These findings highlight the importance of an early diagnosis for proper management of IPF.

Trial registration number: NCT02755441.

Keywords: Interstitial Fibrosis; Rare lung diseases.

Conflict of interest statement

Competing interests: NH reports unrestricted research grants from Aase og Ejnar Danielsens Fond, Roche and Skibsreder Per Henriksen, R. og Hustrus Fond. TSP reports an unrestricted research grant from Boehringer Ingelheim, contact with Galapagos and consulting fees from Boehringer Ingelheim. EB reports payment for presentations from Boehringer Ingelheim, Hofmann la Roche, Galapagos and Astra Zeneca, support for attending meetings from Boehringer Ingelheim and Hofmann la Roche, and participation in advisory boards for Boehringer Ingelheim and Hofmann la Roche. SBS reports payment for presentations from Boehringer Ingelheim and Roche, support for attending meetings from Boehringer Ingelheim, and participation in advisory board for Boehringer Ingelheim.

© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

Figures

Figure 1
Figure 1
Progression-free survival in patients with a short (1 year) diagnostic delay in the entire cohort (A) and stratified according to forced vital capacity (FVC) at the time of diagnosis≤80% predicted (B) or >80% predicted (C).
Figure 2
Figure 2
Unadjusted mean total scores of the SGRQ (A), IPF-specific SGRQ-I derived from the original SGRQ (B), CAT (C) and mean individual score from the CAT questionnaire covering dyspnoea (D) during the first year of follow-up in patients with a short (1 year) diagnostic delay. Higher scores indicate worse quality of life for SGRQ, SGRQ-Ider and CAT. Error bars indicate 95% CIs. CAT, chronic obstructive pulmonary disease (COPD) assessment test; SGRQ, St. George’s Respiratory Questionnaire; SGRQ-Ider, idiopathic pulmonary fibrosis (IPF)-specific SGRQ, derived from the original SGRQ.
Figure 3
Figure 3
Mean all cause hospitalisation and respiratory hospitalisation rates in patients with a short (1 year) diagnostic delay. Error bars indicate 95% CIs.

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