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Research Project

Exacerbations of AATD lung disease: impact and role of augmentation therapy

Principal Investigator:
Alice M. Turner
University of Birmingham
Birmingham, UK
Start date:
January 2022
Contact E-mail:
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Controlling the frequency and severity of exacerbations in AATD, and the management thereof, could have a positive impact on the quality of life, disease progression and prognosis of affected patients. In order to do this we need to better understand pathophysiology of exacerbations of COPD in AATD, and whether conventional treatment is helpful in exacerbation prevention.


  1. Describe the clinical features of exacerbations in patients on and off augmentation in EARCO
  2. Compare exacerbation rate over 12 months between patients on and off augmentation in EARCO
  3. Ascertain if there is an adverse impact of exacerbations on the cardiovascular system in AATD, as measured by incidence of new major cardiovascular event reported by the patient after exacerbations


Inclusion criteria

This study will prospectively collect data on erxacerbations in AATD patients registered to EARCO. Centres will be eligible to take part in this study if they have 10 or more registered patients, whose baseline data for EARCO is complete. Patients will be eligible to take part if they are registered to EARCO, had one or more exacerbations in the year prior to baseline, and consent to the amended study protocol.

Brief summary

Research objective 1 will be completed first, as a descriptive analysis of first exacerbations entered to the study database.

Research objective 2 will be completed using 12 month follow up data and will compare overall rate of exacerbations (primary outcome) between augmented and non-augmented patients, with secondary outcomes of severity of exacerbations (defined by healthcare utilisation) and type (Anthonisen).

Research objective 3 will be carried out using 12 month follow up data and will include an optional biomarker collection attendance at start of exacerbations and 6 weeks after this time point.