Monitoring early lung disease and decline in AATD

  • Research type

    Research Study

  • Full title

    Monitoring early lung disease and decline in alpha-1 antitrypsin deficiency

  • IRAS ID

    311818

  • Contact name

    Alice Turner

  • Contact email

    a.m.turner@bham.ac.uk

  • Sponsor organisation

    University of Birmingham

  • Duration of Study in the UK

    1 years, 9 months, 31 days

  • Research summary

    Patients with alpha-1 antitrypsin deficiency (AATD) experience symptoms due to progresive lung damage. Due to the expense associated with newer treatments for the disease, it is important to identify lung function decline early in these patients. Currently, lung function is monitored using tests such as spirometry. However, these tests are poor at picking up early disease, and only identify a decline once significant damage has already occured in the lungs.

    A pilot study in 2015 identified that some AATD patients with initially normal lung function had abnormal measures of small airways disease. Hence, tests which measure decline of the small airways are likely the most sensitive way to detect early disease changes. Furthermore, tests such as spirometry are dependent on significant effort by the patient; effort independent tests are therefore more helpful in identifying decline as they show less variability.

    The patients from the 2015 pilot had a decline in lung function, measured using standard lung function tools. As it has been almost ten years since generation of these data, this study will aim to restudy the same cohort to determine changes in their health, lung function tests and the measures of small airways dysfunction. This will identify whether the patients with abnormal parameters of small airways have continued to decline, and whether this is reflected in their general health. The aim of this study is to assess progression of early disease in this AATD cohort by comparing lung function tests to health status and a repeat CT scan to determine the sensitivity of these measurements in detecting decline. This will also be correlated with plasma samples taken from the patients both at baseline almost ten years ago, and repeated now.

  • REC name

    West Midlands - Black Country Research Ethics Committee

  • REC reference

    25/WM/0083

  • Date of REC Opinion

    4 Jun 2025

  • REC opinion

    Further Information Favourable Opinion