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

Abstract

<b>Background:</b> 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 a diagnostic delay on progression-free survival, quality of life and hospitalisation rate. <b>Methods:</b> Patients with IPF were included immediately after diagnosis and followed for up to five years with collection of clinical data, St. George9s Respiratory Questionnaires (SGRQ)) and hospitalisation data. Disease progression was defined as decline in FVC &gt; 10% or decline in DLCO &gt; 15%. <b>Results:</b> Among 264 included patients, a diagnostic delay &gt; 1 year was associated with worse progression-free survival compared with a diagnostic delay &lt; 1 year in adjusted analyses (HR: 1.70, 95% CI: 1.18–2.46, p = 0.004), most notably in patients with FVC &gt; 80% predicted at diagnosis (HR: 2.43, 95% CI: 1.45–4.01, p &lt; 0.001). Mean total scores of SGRQ were consistently higher in patients with longer delay, indicating worse quality of life. Mean hospitalisation rates were higher during the first year after diagnosis (IRR: 3.28, 95% CI: 1.35–8.55, p = 0.01) and during the entire follow-up (IRR: 1.74, 95% CI: 1.01–3.02, p = 0.04). <b>Conclusion:</b> A diagnostic delay of more than one year negatively impacts progression-free survival, quality of life and hospitalisation rates in patients with IPF. These findings support the importance of an early diagnosis for proper management of IPF.