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.

3rd Medical Dept Nuremberg General Hospital Paracelsus Medical University Nuremberg Germany

Dept of Genetics and Clinical Immunology National Institute of Tuberculosis and Lung Diseases Warsaw Poland

Dept of Internal Medicine 2 and Pneumology at Wilhelminenspital Wien Vienna Austria

Dept of Internal Medicine Respiratory Disease Unit Spedali Civili Brescia Italy

Dept of Pneumology and Lung Transplantation Bichat Hospital Paris France

Dept of Pneumology Hospital of Santo Andre Centro Hospitalar de Leiria Leiria Portugal

Dept of Pneumology Thomayer Hospital 1st Faculty of Medicine Charles University Prague Czech Republic

Dept of Pulmonology National Koranyi Institute for Pulmonology in Budapest Budapest Hungary

Dept of Respiratory Medicine Beaumont Hospital Royal College of Surgeons in Ireland Dublin Ireland

Dept of Respiratory Medicine Ghent University Hospital Ghent Belgium

Institute of Applied Health Research University of Birmingham Birmingham UK

Leiden University Medical Centre Dept of Pulmonology Leiden Netherlands

Pulmonology Dept Centro Hospitalar de São João Porto Portugal

Pulmonology Dept University Hospital Vall d'Hebron CIBER de Enfermedades Respiratorias Barcelona Spain

Pulmonology. 2020 Jul - Aug;26(4):181-183. doi: 10.1016/j.pulmoe.2020.01.003. PubMed

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