TY - JOUR T1 - Diagnosis and management of α<sub>1</sub>-antitrypsin deficiency in Europe: an expert survey JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00171-2018 VL - 5 IS - 1 SP - 00171-2018 AU - Ildikó Horváth AU - Maria Canotilho AU - Jan Chlumský AU - Joanna Chorostowska-Wynimko AU - Luciano Corda AU - Eric Derom AU - Joachim H. Ficker AU - Meinhard Kneussl AU - Marc Miravitlles AU - Maria Sucena AU - Gabriel Thabut AU - Alice M. Turner AU - Emily van ’t Wout AU - N. Gerard McElvaney Y1 - 2019/02/01 UR - http://openres.ersjournals.com/content/5/1/00171-2018.abstract N2 - Despite recent improvements, α1-antitrypsin deficiency (AATD) remains a rarely diagnosed and treated condition. To assess the variability of AATD diagnosis/treatment in Europe, and to evaluate clinicians’ views on methods to optimise management, specialist AATD clinicians were invited to complete a web-based survey.Surveys were completed by 15 physicians from 14 centres in 13 European countries. All respondents perceived the AATD diagnosis rate to be low in their country; 77% of physicians believed that ∼15% of cases were diagnosed. Low awareness was perceived as the greatest barrier to diagnosis. Spirometry was considered more practical than quantitative computed tomography (QCT) for monitoring AATD patients in clinical practice; QCT was considered more useful in trials. AAT therapy provision was reported to be highly variable: France and Germany were reported to treat the highest proportion (∼60%) of diagnosed patients, in contrast to the UK and Hungary, where virtually no patients receive AAT therapy. Most clinicians supported self-administration and extended dosing intervals to improve convenience of AAT therapy.This survey indicates that AATD diagnosis and management are highly heterogeneous in Europe; European cooperation is essential to generate data to support access to AAT therapy. Improving convenience of AAT therapy is an ongoing objective.Access to α1-antitrypsin (α1-AT) therapy varies in Europe; where available, α1-AT therapy optimisation is the goal http://ow.ly/YL6m30n4LV3 ER -