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 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.
CommentsThe original article is available at openres.ersjournals.com
Published CitationHorváth 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
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