Idiopathic pulmonary fibrosis (IPF) is a chronic condition of unknown cause resulting in scarring of the lungs. It is more common in men, with older age and smoking being additional risk factors. Currently, there are no treatments available that can cure the disease, only medications that can slow the rate of scarring. IPF has a very poor prognosis, with the average patient surviving only 3-4 years post diagnosis, an outlook which is worse than several cancers.
Most patients with IPF tend to have several other medical problems which affect both the lungs and other organs. Conditions affecting the heart tend to be the most common medical problem. However, while studies have shown that some heart conditions such as coronary heart disease and right heart failure occur frequently in IPF, we do not know to what extent left-sided heart failure affects those with IPF and also whether treating heart failure affects survival in these patients.
The aim of this proposed study is to use CPRD Aurum data to determine the trends in both existing and new cases of left-sided heart failure in patients with IPF over a 10-year period as well as determine the effect that heart failure treatment may have on the risk of death in IPF. Determining this would help guide clinical management and prognosis in patients with both of these diseases.
Idiopathic pulmonary fibrosis (IPF) is a progressive lung condition of unknown aetiology, resulting in irreversible lung scarring. Multimorbidity is common in IPF and a risk factor for reduced survival. In particular, cardiovascular diseases are the most prevalent comorbidity and significant risk factors for increased mortality.
Nevertheless, while data are available on ischaemic heart disease and pulmonary hypertension with associated right-heart failure, no data are available on the incidence or prevalence of left-sided heart failure within this patient population, nor are there any data on the impact of heart failure treatments on IPF survival.
Therefore, we aim to conduct a retrospective cohort study using CPRD Aurum data to assess trends in incidence and prevalence of left-sided heart failure in IPF patients and the impact of heart failure treatments on IPF mortality over a 10-year period between 2010 – 2019. The study population will include adults aged >40 years with a SNOMED-CT code denoting an IPF diagnosis, with 12 months’ worth of registration data prior to cohort entry and a HES and ONS-linked record. Baseline demographics, cardiovascular risk factors and cardiovascular medication use will be described. Heart failure prevalence and incidence in the cohort will be calculated, with heart failure identified by presence of a code denoting a heart failure diagnosis and validated algorithms to differentiate between heart failure with preserved or reduced ejection fraction. Calculated heart failure prevalence and incidence from this IPF cohort will be compared to published estimates in the general population and COPD cohorts. Subsequently, using ONS-linked data, a multivariate Cox proportional hazards regression model will be fitted using stepwise selection to determine adjusted hazard ratios for the risk of incident (<6 months) or prevalent (>6 months) use of the following heart failure treatments - ARBs, ACEi, beta-blockers, loop diuretics and MRAs -on all-cause, IPF-specific and cardiovascular mortality.
Incidence of left-sided heart Failure (HF) in IPF patients, stratified by age, gender and calendar year; Prevalence of left-sided heart failure in IPF patients, stratified by age, gender and calendar year; Crude and adjusted hazard ratios of the risk between incident (< 6months) and prevalent (> 6 months) use of each of the following heart failure treatments: angiotensin receptor blockers (ARBs), angiotensin converting enzyme inhibitors (ACEi), beta-blockers, loop diuretics and mineralocorticoid receptor antagonists (MRA), on all-cause, IPF-specific and cardiovascular mortality between 2010 – 2019
Jennifer Quint - Chief Investigator - Imperial College London
Ardita Koteci - Corresponding Applicant - Imperial College London
Ann Morgan - Collaborator - Imperial College London
Hannah Whittaker - Collaborator - Imperial College London
Laura Portas - Collaborator - Imperial College London
Peter George - Collaborator - Royal Brompton Hospital
HES Admitted Patient Care;ONS Death Registration Data;Patient Level Index of Multiple Deprivation