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

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Abstract
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.

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MLA
Horvath, Ildikó et al. “Diagnosis and Management of Α1-antitrypsin Deficiency in Europe : an Expert Survey.” ERJ OPEN RESEARCH 5.1 (2019): n. pag. Print.
APA
Horvath, Ildikó, Canotilho, M., Chlumský, J., Chorostowska-Wynimko, J., Corda, L., Derom, E., Ficker, J. H., et al. (2019). Diagnosis and management of α1-antitrypsin deficiency in Europe : an expert survey. ERJ OPEN RESEARCH, 5(1).
Chicago author-date
Horvath, Ildikó, Maria Canotilho, Jan Chlumský, Joanna Chorostowska-Wynimko, Luciano Corda, Eric Derom, Joachim H Ficker, et al. 2019. “Diagnosis and Management of Α1-antitrypsin Deficiency in Europe : an Expert Survey.” Erj Open Research 5 (1).
Chicago author-date (all authors)
Horvath, Ildikó, Maria Canotilho, Jan Chlumský, Joanna Chorostowska-Wynimko, Luciano Corda, Eric Derom, Joachim H Ficker, Meinhard Kneussl, Marc Miravitlles, Maria Sucena, Gabriel Thabut, Alice Turner, Emily van  ’t Wout, and N Gerard McElvaney. 2019. “Diagnosis and Management of Α1-antitrypsin Deficiency in Europe : an Expert Survey.” Erj Open Research 5 (1).
Vancouver
1.
Horvath I, Canotilho M, Chlumský J, Chorostowska-Wynimko J, Corda L, Derom E, et al. Diagnosis and management of α1-antitrypsin deficiency in Europe : an expert survey. ERJ OPEN RESEARCH. 2019;5(1).
IEEE
[1]
I. Horvath et al., “Diagnosis and management of α1-antitrypsin deficiency in Europe : an expert survey,” ERJ OPEN RESEARCH, vol. 5, no. 1, 2019.
@article{8610527,
  abstract     = {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.},
  articleno    = {00171-2018},
  author       = {Horvath, Ildikó and Canotilho, Maria and Chlumský, Jan and Chorostowska-Wynimko, Joanna and Corda, Luciano and Derom, Eric and Ficker, Joachim H and Kneussl, Meinhard and Miravitlles, Marc and Sucena, Maria and Thabut, Gabriel and Turner, Alice and van 't Wout, Emily and McElvaney, N Gerard},
  issn         = {2312-0541},
  journal      = {ERJ OPEN RESEARCH},
  language     = {eng},
  number       = {1},
  pages        = {11},
  title        = {Diagnosis and management of α1-antitrypsin deficiency in Europe : an expert survey},
  url          = {http://dx.doi.org/10.1183/23120541.00171-2018},
  volume       = {5},
  year         = {2019},
}

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