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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorGreulich, Timm
dc.contributor.authorAlbert, Anna
dc.contributor.authorCassel, Werner
dc.contributor.authorBoeselt, Tobias
dc.contributor.authorPeychev, Erika
dc.contributor.authorKlemmer, Andreas
dc.contributor.authorMiravitlles Fernández, Marc
dc.date.accessioned2022-08-10T10:22:52Z
dc.date.available2022-08-10T10:22:52Z
dc.date.issued2022-01-05
dc.identifier.citationGreulich T, Albert A, Cassel W, Boeselt T, Peychev E, Klemmer A, et al. Opinions and Attitudes of Pulmonologists About Augmentation Therapy in Patients with Alpha-1 Antitrypsin Deficiency. A Survey of the EARCO Group. Int J Chron Obstruct Pulmon Dis. 2022 Jan 5;17:53–64.
dc.identifier.issn1178-2005
dc.identifier.urihttp://hdl.handle.net/11351/7980
dc.descriptionEARCO; Augmentation therapy; Survey
dc.description.abstractBackground: Augmentation therapy (AT) is the only specific treatment licensed for patients with alpha-1 antitrypsin deficiency (AATD) associated lung disease. Since patients with severe AATD may have a very different prognosis and AT requires intravenous infusions for life, the decision to initiate AT may be challenging. Methods: This survey was conducted on 63 experts in AATD from 13 European countries about their opinions and attitudes regarding AT. Participants were asked to rank the importance of 11 identified factors related with the prescription of AT. In addition, each participant was asked to respond to the indication of AT for 30 out of 500 hypothetical cases developed with the combinations of the 11 factors. Each case was evaluated by 3 experts to check the concordance. Results: The variables that scored higher on preferences for initiating AT were AAT genotype (score 8.6 from a Likert scale 0– 10 (SD: 1.7)), AATD serum level (8.2 (SD:2.4)) and FEV1 (%) decline (7.9 (SD:2.4)). Among the 500 different cases, there was an agreement in indication of AT among the 3 experts in 291 (58.2%). Regarding the variables associated with AT, it was indicated to 81.9% of Pi*ZZ, 52.4% of Pi*SZ and 9.8% of Pi*MZ (p < 0.0001). For Pi*ZZ patients, multivariate analysis identified younger age, reduced FEV1 (%), higher FEV1 decline and worse emphysema as significantly associated with prescription (AUC = 0.8114); for Pi*SZ variables were younger age, worse FEV1 (%) and worse emphysema (AUC = 0.7414); and for Pi*MZ younger age, worse DLCO (%), higher DLCO decline and dyspnea (AUC = 0.8387). Conclusion: There is a high variability in the criteria for prescription of AT among European experts. Most cases were recommended AT according to guidelines, but a significant number of patients with genotype Pi*SZ and almost 10% Pi*MZ were recommended to initiate AT despite the lack of evidence of efficacy in these genotypes.
dc.language.isoeng
dc.publisherDove Medical Press
dc.relation.ispartofseriesInternational Journal of Chronic Obstructive Pulmonary Disease;17
dc.rightsAttribution-NonCommercial 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by-nc/4.0/
dc.sourceScientia
dc.subjectPulmons - Malalties obstructives - Tractament
dc.subjectEnzims proteolítics - Inhibidors
dc.subject.meshalpha 1-Antitrypsin Deficiency
dc.subject.mesh/drug therapy
dc.subject.meshPulmonary Disease, Chronic Obstructive
dc.titleOpinions and Attitudes of Pulmonologists About Augmentation Therapy in Patients with Alpha-1 Antitrypsin Deficiency. A Survey of the EARCO Group
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.2147/COPD.S346051
dc.subject.decsdeficiencia de alfa 1-antitripsina
dc.subject.decs/farmacoterapia
dc.subject.decsenfermedad pulmonar obstructiva crónica
dc.relation.publishversionhttps://doi.org/10.2147/COPD.S346051
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[Greulich T, Albert A, Boeselt T, Peychev E, Klemmer A] University Medical Centre Giessen and Marburg, Philipps-University, Department of Medicine, Pulmonary and Critical Care Medicine, Member of the German Centre for Lung Research (DZL), Marburg, Germany. [Cassel W] University Medical Centre Giessen and Marburg, Philipps-University, Department of Medicine, Pulmonary and Critical Care Medicine, Sleep Disorders Centre, Member of the German Centre for Lung Research (DZL), Marburg, Germany. [Miravitlles M] Servei de Pneumologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. CIBER de Enfermedades Respiratorias (CIBERES), Barcelona, Spain
dc.identifier.pmid35023913
dc.identifier.wos000742383900001
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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