RT Journal Article
SR Electronic
T1 Baseline characteristics of patients enrolled in the EARCO prospective registry of alpha-1 antitrypsin deficiency: preliminary results
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA1834
DO 10.1183/13993003.congress-2021.PA1834
VO 58
IS suppl 65
A1 Alice Turner
A1 Maria Torres
A1 José Luis López-Campos
A1 Catarina Guimaraes
A1 Joana Chorostowska-Wynimko
A1 Robert Bals
A1 Ilaria Ferrarotti
A1 Gerard Mcelvaney
A1 Karen O’Hara
A1 Jan Stolk
A1 Robert Stockley
A1 Marion Wilkens
A1 Christian Clarenbach
A1 Timm Greulich
A1 Marc Miravitlles
YR 2021
UL http://erj.ersjournals.com/content/58/suppl_65/PA1834.abstract
AB The European Alpha-1 antitrypsin deficiency Research Collaboration (EARCO) is a Clinical Research Collaboration (CRC) of the ERS with the main objective to develop a prospective registry of individuals with the deficiency to understand the natural history of the disease and the impact of therapies better. We aim to describe the baseline characteristics of patients enrolled during the first year of the registry.The registry (http://earco.estudinord.com) was launched on February 2020 and consists of baseline and yearly follow-up data. By February 2021 there were 34 centers from 10 countries ready to enroll and the data base included information from 235 patients from 5 countries. Phenotypes were: 110 Pi*ZZ, 79 Pi*SZ, 16 Pi*SS and 30 others. Globally, 54.5% were male, 75% index cases, with a mean age of 54.3 years (SD:14.8), 66% with lung and 9.8% with liver disease. Mean FEV1= 78% pred. (SD:32.4%) and KCO 76% (SD:22%), with a mean CAT of 11 (SD:8) and BODEx index of 1.3 (SD:1.8). Among the 110 Pi*ZZ individuals, the mean age was 56.1 years (SD: 12.7), 90% with lung and 15% with liver disease; mean FEV1= 65.4% (SD:31.6%) and KCO 69% (SD:20%) with a mean CAT of 13.6 (SD:8) and BODEx index of 2 (SD: 2), 66 (61%) of Pi*ZZ patients and 6 patients with other genotypes were on augmentation therapy.Despite the difficulties with the different interpretations of data protection laws across countries and the slow recruitment due to the COVID-19 pandemic, the EARCO group has been able to engage a large number of centers from 10 countries and demonstrated to be a valid platform to collect standardized prospective data from patients with AATD.FootnotesCite this article as: European Respiratory Journal 2021; 58: Suppl. 65, PA1834.This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).