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Research Project

Vasculitis in Individuals with Alpha-1 Antitrypsin Deficiency: A Case-Control Study using the EARCO Registry.

Principal Investigator:
Reem Alluhibi
Center:
University College London
City/Country:
London, UK
Start date:
November 2024
Status:
Ongoing
Contact E-mail:
reem.alluhibi.23@ucl.ac.uk
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Introduction

Several studies have suggested a connection between AATD and granulomatosis with polyangiitis (GPA) formerly known as Wegener's granulomatosis (WG).

Carriage of the S and Z alleles is increased in ANCA-associated vasculitis (AAV), with 5–27% of patients with GPA carrying the Z allele. Moreover, Deshayes et al found that GPA patients with S allele displayed a higher frequency of pulmonary involvement than other GPA patients. Although both conditions involve significant respiratory complications, little is known about how the presence of GPA in individuals with AATD is associated with lung and liver disease severity.

Objectives

We aim to address the gap in understanding the impact of GPA on lung status in individuals with AATD by conducting a case-control study using data from the EARCO registry.

Inclusion criteria

The study will include all individuals with both AATD and GPA as cases, and the control group will consist of three individuals for each case matched on age, sex, and genotype with AATD but without GPA

Brief summary

The primary analysis will focus on lung function parameters (FEV1, FVC, and DLCO) and clinical outcomes, including hospitalization rates and exacerbation frequency. Secondary outcomes will include quality of life assessments using CAT and EQ-5D Questionnaires and liver assessments.

When longitudinal data are available, we will assess time to exacerbation or hospitalization and change in lung function.

 

This study is expected to clarify whether individuals with AATD and GPA have worse lung function, higher hospitalization rates, and more frequent exacerbations compared to those with AATD alone, while also providing insight into the effects on quality of life, and other comorbid conditions.