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Research Project

Prognosis of co-existent lung and liver disease in alpha-1 antitrypsin deficiency

Principal Investigator:
Alice M. Turner
University of Birmingham
Birmingham, UK
Start date:
October 2021
Contact E-mail:
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Whilst augmentation therapy may ameliorate lung disease progression, this has not been proven physiologically in trials, though radiological trial data is highly supportive. As new drugs become available which target the liver and reduce AAT level it becomes important to identify whether those with liver disease differ in progression from those without prior to receiving this type of treatment. It is possible that the presence of liver disease generates systemic inflammation, which could be detrimental to the lung in AATD, given the greater degree of protease imbalance that would occur. Systemic inflammation might also drive liver disease progression and decompensation, and is known to occur with COPD, and associate with poor outcomes. This makes understanding the potential synergy between liver and disease important in AATD.


Investigate the prognosis of patients with AATD according to presence and severity of lung and liver disease.

Inclusion criteria

Patients with severe AATD included in the EARCO registry

Brief summary

This project has two parts

  1. A prospective element will use data from all centers participating, analysing data collected within the main EARCO database. 
  2. A retrospective part for which all EARCO centers who have a preceding national (or other type of) registry which collected data on presence or absence of liver disease and outcome data.