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Diagnosis and management of alpha1-antitrypsin deficiency in Europe: an expert survey Horváth, Ildiko Canotilho, Maria Chlumský, Jan Chorostowska-Wynimko, Joanna Corda, Luciano Derom, Eric Miravitlles Fernández, Marc Institut Català de la Salut [Horváth I] Department of Pulmonology, National Koranyi Institute for Pulmonology in Budapest, Budapest, Hungary. [Canotilho M] Department of Pneumology, Hospital of Santo Andre - Centro Hospitalar de Leiria, Leiria, Portugal. [Chlumský J] Department of Pneumology, Thomayer Hospital Prague, Czech Republic. First Faculty of Medicine, Charles University, Prague, Czech Republic. [Chorostowska-Wynimko J] Department of Genetics and Clinical Immunology, National Institute of Tuberculosis and Lung Diseases, Warsaw, Poland. [Corda L] Department of Internal Medicine, Respiratory Disease Unit, Spedali Civili, Brescia, Italy. [Derom E] Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium. [Miravitlles M] Servei de Pneumologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain. CIBER de Enfermedades Respiratorias (CIBERES), Barcelona, Spain. Vall d'Hebron Barcelona Hospital Campus Malalties congènites - Diagnòstic Pulmons - Malalties - Europa DISEASES::Respiratory Tract Diseases::Lung Diseases::alpha 1-Antitrypsin Deficiency Other subheadings::Other subheadings::/diagnosis GEOGRAPHICALS::Geographic Locations::Europe Other subheadings::Other subheadings::Other subheadings::/epidemiology ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::deficiencia de alfa 1-antitripsina Otros calificadores::Otros calificadores::/diagnóstico DENOMINACIONES GEOGRÁFICAS::localizaciones geográficas::Europa (continente) Otros calificadores::Otros calificadores::Otros calificadores::/epidemiología AATD deficiency; Diagnosis; Europe Deficiència d'AATD; Diagnosi; Europa Deficiencia de AATD; Diagnóstico; Europa 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. 2019-05-03T10:38:28Z 2019-05-03T10:38:28Z 2019-03-11 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Horváth I, Canotilho M, Chlumský J, Chorostowska-Wynimko J, Corda L, Derom E, et al. Diagnosis and management of α 1 -antitrypsin deficiency in Europe: an expert survey. ERJ Open Res. 2019;5(1):00171–2018. 2312-0541 https://hdl.handle.net/11351/4055 10.1183/23120541.00171-2018 30863774 http://hdl.handle.net/11351/4055 eng ERJ Open Research;5(1) https://openres.ersjournals.com/content/5/1/00171-2018 Attribution-NonCommercial-NoDerivatives 4.0 International http://creativecommons.org/licenses/by-nc-nd/4.0/ info:eu-repo/semantics/openAccess application/pdf European Respiratory Society Scientia