Acute exacerbation of idiopathic pulmonary fibrosis in the UK: incidence, mortality and disease severity

Date of ISAC Approval: 
Lay Summary: 
Idiopathic pulmonary fibrosis (IPF) is a rare lung disease that worsens over time, and has an average life expectancy of 3-5 years once diagnosed. The course of the disease is variable and unpredictable. Patients may experience sudden onset of worsened symptoms (e.g. shortness of breath) for which no other cause can be found; these events are referred to as 'acute exacerbations'. They can lead to decreased lung function, admission to hospital and even death. It is not clear how these events impact on health resources. Therefore, the aim of this project is to estimate the incidence of acute exacerbations in patients with IPF as well as the associated death and hospitalisation rates, in order to evaluate the impact on health care resources in the UK.
Technical Summary: 
IPF population from CPRD will be linked with HES in order to determine eligible patients for our proposed study population. Descriptive statistics (counts and percentages for categorical variables; and mean, range and SD for continuous variables (additionally median, IQR for non-normally distributed variables)) will be generated to describe the cohort of IPF patients at diagnosis in relation to age (continuous and categorical data), sex, BMI (continuous and categorical data) and year of diagnosis. Patients' pre-diagnosis history will be assessed for the period preceding IPF diagnosis to the date of registration with the clinical practice. Descriptive statistics will be generated to describe the most common medical conditions experienced by patients prior to IPF diagnosis. Finally, FVC will be categorized in three groups at diagnosis (<50% predicted, 50%-80% predicted, >80% predicted). FVC at diagnosis will be defined as the first FVC measurement between diagnosis and 90 day period post diagnosis. If not available, FVC at diagnosis will be defined as the latest FVC closest to the diagnosis date.
Health Outcomes to be Measured: 
Incidence rate of acute exacerbation in IPF patients Mortality associated with acute exacerbation in IPF patients Hospitalisation associated with acute exacerbation in IPF patients

Michael Marcus - Chief Investigator - Boehringer-Ingelheim - UK
Dr Chris Poole - Collaborator - Boehringer-Ingelheim - UK
Dr Deborah Clarke - Collaborator - Boehringer-Ingelheim - UK
Michael Marcus - Corresponding Applicant - Boehringer-Ingelheim - UK
Prithwiraj Das - Collaborator - Boehringer-Ingelheim - UK
Dr Sophie Fletcher - Collaborator - University Hospital Southampton

HES Admitted;HES Outpatient;ONS