TY - JOUR T1 - Protocol for the EARCO Registry: a pan-European observational study in patients with α<sub>1</sub>-antitrypsin deficiency JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00181-2019 VL - 6 IS - 1 SP - 00181-2019 AU - Timm Greulich AU - Alan Altraja AU - Miriam Barrecheguren AU - Robert Bals AU - Jan Chlumsky AU - Joanna Chorostowska-Wynimko AU - Christian Clarenbach AU - Luciano Corda AU - Angelo Guido Corsico AU - Ilaria Ferrarotti AU - Cristina Esquinas AU - Caroline Gouder AU - Ana Hećimović AU - Aleksandra Ilic AU - Yavor Ivanov AU - Sabina Janciauskiene AU - Wim Janssens AU - Malcolm Kohler AU - Alvils Krams AU - Beatriz Lara AU - Ravi Mahadeva AU - Gerry McElvaney AU - Jean-François Mornex AU - Karen O'Hara AU - David Parr AU - Eava Piitulainen AU - Karin Schmid-Scherzer AU - Niels Seersholm AU - Robert A. Stockley AU - Jan Stolk AU - Maria Sucena AU - Hanan Tanash AU - Alice Turner AU - Ruxandra Ulmeanu AU - Marion Wilkens AU - Arzu Yorgancioğlu AU - Ana Zaharie AU - Marc Miravitlles A2 - , Y1 - 2020/01/01 UR - http://openres.ersjournals.com/content/6/1/00181-2019.abstract N2 - Rationale and objectives Alpha-1 antitrypsin deficiency (AATD) is a genetic condition that leads to an increased risk of emphysema and liver disease. Despite extensive investigation, there remain unanswered questions concerning the natural history, pathophysiology, genetics and the prognosis of the lung disease in association with AATD. The European Alpha-1 Clinical Research Collaboration (EARCO) is designed to bring together researchers from European countries and to create a standardised database for the follow-up of patients with AATD.Study design and population The EARCO Registry is a non-interventional, multicentre, pan-European, longitudinal observational cohort study enrolling patients with AATD. Data will be collected prospectively without interference/modification of patient's management by the study team. The major inclusion criterion is diagnosed severe AATD, defined by an AAT serum level &lt;11 µM (50 mg·dL−1) and/or a proteinase inhibitor genotype ZZ, SZ or compound heterozygotes or homozygotes of other rare deficient variants. Assessments at baseline and during the yearly follow-up visits include lung function testing (spirometry, body plethysmography and diffusing capacity of the lung), exercise capacity, blood tests and questionnaires (symptoms, quality of life and physical activity). To ensure correct data collection, there will be designated investigator staff to document the data in the case report form. All data will be reviewed by the EARCO database manager.Summary The EARCO Registry aims to understand the natural history and prognosis of AATD better with the goal to create and validate prognostic tools to support medical decision-making.The EARCO Registry is a non-interventional, multicentre, pan-European, longitudinal observational cohort study enrolling patients with AATD to elucidate the natural history, pathophysiology, genetics and prognosis of this condition http://bit.ly/369ScCc ER -