Burden of idiopathic pulmonary fibrosis in the united kingdom using electronic health records

This thesis aims to deepen the understanding of the burden of ILD, with a particular focus on its most aggressive subtype, IPF, by addressing gaps in the current epidemiological knowledge. The primary objectives were to estimate the incidence, prevalence, and mortality of IPF and its subtypes in England, and to investigate potential changes in the disease's epidemiology over the past decade, considering advances in diagnostic techniques and the availability of treatment options. This research includes an assessment of the reliability of IPF diagnostic coding in UK primary care, an update on the epidemiology of IPF from 2008 to 2018, and an exploration of trends in survival and causes of death in patients, comparing the pre- and post-antifibrotic eras. The study found that diagnostic coding for IPF in primary care has become more focused over time, with a noticeable increase in the incidence of IPF defined using diagnostic codes in primary care settings, suggesting improved management in primary setting. However, hospital-based data showed a gradual decrease in cases, potentially reflecting better management and earlier diagnosis in primary care, reducing the need for intervention for IPF related complications. In terms of IPF-related mortality, the data revealed an initial increase, followed by a stable rate towards the end of the study period, though the lack of follow-up data makes it difficult to draw firm conclusions regarding its impact. Similarly, survival rates for IPF patients showed no significant improvement, which could be due to factors such as eligibility and access of antifibrotic treatments, delays in initiating treatment, or the advanced stage of disease at the time of diagnosis. Overall, the findings of this thesis contribute to a clearer understanding of the evolving burden of IPF in England, with implications for guiding more effective care strategies for patients with this chronic and debilitating disease.