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

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posted on 22.11.2019 by 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, Noel G. McElvaney

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.

Funding

CSL Behring.

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The original article is available at openres.ersjournals.com

Published Citation

Horváth I, Canotilho M, Chlumský , Chorostowska-Wynimko J, Corda L, Derom E, Ficker JH, Kneussl M, Miravitlles M, Sucena M, Thabut G, Turner AM, van 't Wout E, McElvaney NG. Diagnosis and management of α1-antitrypsin deficiency in Europe: an expert survey. ERJ Open Research. 2019;5(1). pii: 00171-2018

Publication Date

01/02/2019

Publisher

European Respiratory Society

PubMed ID

30863774

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