Diagnosis and management of α1-antitrypsin deficiency in Europe: an expert survey

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...

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Bibliographic Details
Main Authors: Ildikó Horváth, Maria Canotilho, Jan Chlumský, Joanna Chorostowska-Wynimko, Luciano Corda, Eric Derom, Joachim H. Ficker, Meinhard Kneussl, Marc Miravitlles, Maria Sucena, Gabriel Thabut, Alice M. Turner, Emily van ’t Wout, N. Gerard McElvaney
Format: Article
Language:English
Published: European Respiratory Society 2019-03-01
Series:ERJ Open Research
Online Access:http://openres.ersjournals.com/content/5/1/00171-2018.full
Description
Summary: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.
ISSN:2312-0541