Episodes of acute respiratory deterioration in patients with idiopathic pulmonary fibrosis in the UK – mortality and contributing factors

Marcus, Michael, Clarke, Deborah, Tebboth, Abby, Das, Prithwiraj and Fletcher, Sophie (2019) Episodes of acute respiratory deterioration in patients with idiopathic pulmonary fibrosis in the UK – mortality and contributing factors. European Respiratory Journal, 54 (Suppl 63), [PA1355]. (doi:10.1183/13993003.congress-2019.PA1355).

Abstract

Episodes of acute respiratory deterioration (ARD) in idiopathic pulmonary fibrosis are associated with significant morbidity and mortality. The incidence and potential associated risk factors for these episodes remains unknown. This large retrospective study provides an estimate of the incidence, mortality and factors associated with these episodes in patients with IPF in the UK. Linked routinely-collected primary care data on 5,276 patients diagnosed with IPF between 2000 and 2014 were used. Incidence and mortality of ARD episodes were estimated using Poisson and Cox regression methods respectively. 4.2% of patients with IPF had an ARD episode over the study period. The overall incident rate was 11.6 per 10,000 patient years (95%CI 10.1-13.2). The overall year-on-year increase in mortality was ~39% (rate ratio 1.39, 95% CI 1.21-1.60). Age, COPD, death and hospital admission were significantly associated with incidence of ARD. The number of exacerbation episodes i.e. one, two and three exacerbations and hospital admission were associated with increased mortality. Episodes of ARD in patients with IPF are significantly associated with mortality. Mortality is also associated with male sex and increasing age. The overall incidence of episodes of ARD in this large study increased between 2000 and 2014 while survival got worse. COPD was an associated risk factor of ARD. The findings of this study are consistent with clinical experience but do require a further prospective validation cohort study.

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