Fibrosing lung diseases: determinants, prognosis, and disease prediction

Study type
Protocol
Date of Approval
Study reference ID
18_291
Lay Summary

Fibrosing lung diseases (FLD) are a group of chronic diseases that shrink and stiffen the lungs. FLD patients can live for an only short period (3-5 years) from diagnosis, and case numbers have been increasing every year. Moreover, FLD is often misdiagnosed and managed inappropriately resulting in a higher death rate. This study aims to examine FLD patients, with particular emphasis on idiopathic pulmonary fibrosis (IPF): the family of lung diseases characterised by abnormal thickening and stiffness of lung tissue that is not due to infection or cancer, by systematically extracting information on diagnoses, prescriptions, patientsÂ’ characteristics and symptoms recorded in general practices (GPs), hospitals, and death registry. We will include patients aged 40 years and older without a history of FLD and follow them up until they transfer out of their GPs, have an FLD diagnosis, die, or end of study period, whichever occurs first. We will further monitor these patients for disease progression. Follow-up data will examine various issues, including 1) what are the risk factors of disease, 2) how is disease progressing over time, 3) which medications can lower risk of disease, and 4) how can we predict who will develop the disease and how long they can survive after diagnosis. Findings from this study can improve our understanding of FLD, improve quality of care and management, and provide insight into preventive strategies for FLD.

Technical Summary

Fibrosing lung diseases (FLD) is a group of chronic, progressive, fibrotic lung diseases with unknown aetiology associated with a shortened survival rate, and its incidence has been increasing every year. It is often misdiagnosed and managed inappropriately resulting in poor outcomes. This study aims to utilise linkage data from GPs, hospitals, and death registry to develop an algorithm to identify patients with FLD, with particular emphasis on Idiopathic Pulmonary Fibrosis (IPF), based on their diagnostic codes, symptoms, characteristics, and prescribed medications. Patients aged 40 years and older who did not have a history of FLD will be initially included and followed-up until they were censored (i.e., transfer out of their GP, being diagnosed, deaths, or end of study period). Then FLD cases will be monitored for disease progression (i.e., hospitalisation, development of lung cancer, or death), which leads to further investigation on the associations between disease and various risk factors, patterns of disease progression, medication options that might potentially be linked with disease risk, and models to predict FLD onset and prognosis. Findings from this study can improve understanding of the natural history of FLD, improve quality of care and management, and provide insight into preventive strategies.

Health Outcomes to be Measured

Newly diagnosed fibrosing lung disease (FLD)- Newly diagnosed idiopathic pulmonary fibrosis (IPF)
- Newly diagnosed lung cancer
- Hospitalisation from any causes
- Respiratory mortality
- All-cause mortality

Collaborators

Harry Hemingway - Chief Investigator - University College London ( UCL )
Burcu Ozaltin - Corresponding Applicant - University College London ( UCL )
Arturo Gonzalez-Izquierdo - Collaborator - University College London ( UCL )
Athol Wells - Collaborator - Royal Brompton Hospital
Joanna Porter - Collaborator - University College London ( UCL )
Joseph Jacob - Collaborator - University College London ( UCL )
Kenan Direk - Collaborator - University College London ( UCL )
Mark Jones - Collaborator - University of Southampton
Muhammad Qummer ul Arfeen - Collaborator - University College London ( UCL )
Nat Na-Ek - Collaborator - Farr Institute of Health Informatics Research
Spiros Denaxas - Collaborator - University College London ( UCL )

Linkages

HES Accident and Emergency;HES Admitted Patient Care;ONS Death Registration Data;Patient Level Index of Multiple Deprivation