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Research Project

FLARE = Future of aLpha-1 AntitRypsin dEficiency =Prediction Of Mortality Or Lung Transplantation In Patients With AATD

Principal Investigator:
Arthur Pavot
Center:
Bordeaux
City/Country:
Bordeaux
Start date:
March 2024
Status:
Ongoing
Contact E-mail:
pavot.arthur@gmail.com
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Introduction

Emphysema secondary to alpha-1-anti-trypsin deficiency (AATD) is a rare disease but pejorative event such as death or lung transplantation seem to affect up to 15.6% of patients. Hence, predicting these adverse outcomes is a major challenge.

We therefore aim to develop prediction model for mortality or lung transplantation to identify at-risk patients, combining clinical, biological, functional and quantitative CT characteristics (including radiomics), for patients with emphysema secondary to AATD.

Objectives

Development of a prediction model for death or lung transplantation to identify at-risk patients would allow to identify patients with AATD at risk of clinical deterioration, in order to

  1. anticipate registration on the transplant list,
  2. optimize overall management, including drug and non-drug management.

 

Inclusion criteria

Patients over 18 years old will be included retrospectively from 2010 if they have had a chest CT. The next step of our project would be to validate our results on a larger scale using data from the European EARCO cohort.

Brief summary

AATD is a rare disease which can lead to adverse events such as death or lung transplantation.  Predicting these events is a major challenge for AATD patients.

Clinical prognostic scores exist concerning overall COPD patients but they have limitations, especially concerning AATD. We plan to develop an innovative tool including AI-driven analysis of thoracic CT-scans in addition to usual clinical features. This tool could allow us to identify patients with AATD at risk of clinical deterioration in order to anticipate registration on transplantation list and optimize overall management, including drug and non-drug.