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Long-term experience in the treatment of α1-antitrypsin deficiency: 25 years of augmentation therapy

Helmut Teschler
European Respiratory Review 2015 24: 46-51; DOI: 10.1183/09059180.10010714
Helmut Teschler
Dept of Respiratory Medicine, West German Lung Clinic, Essen University Hospital, Essen, Germany
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  • For correspondence: Helmut.Teschler@Ruhrlandklinik.UK-Essen.de
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Abstract

Although it is often under-recognised, α1-antitrypsin deficiency (AATD) represents one of the most common genetic respiratory disorders worldwide. Since the publication of studies in the late 1980s, which demonstrated that plasma-derived augmentation therapy with intravenous α1-antitrypsin (AAT) can reverse the biochemical deficiencies in serum and lung fluid that characterise emphysema, augmentation therapy has become the cornerstone of patient management. This article, with a focus on experience gained in clinical practice in Germany, provides an overview of some of the research highlights and clinical experience gained in the use of augmentation therapy for AATD during the past 25 years, and briefly discusses the potential role of AAT augmentation therapy in lung transplant recipients. Additionally, the goals of AAT augmentation therapy will be discussed, namely to delay the progression of emphysema, reduce the frequency of exacerbations and improve health-related quality of life. Beyond pulmonary disease, there is recent growing evidence to indicate that AATD could also play a role in rare disorders such as panniculitis, granulomatosis with polyangiitis and ulcerative colitis.

Abstract

Overview of long-term experience gained with AAT augmentation therapy for the treatment of AATD http://ow.ly/HmJLR

Footnotes

  • Conflict of interest: Disclosures can be found alongside the online version of this article at err.ersjournals.com

  • Provenance: Publication of this peer-reviewed article was supported by Grifols, Barcelona, Spain (article sponsor, European Respiratory Review issue 135).

  • Received November 13, 2014.
  • Accepted December 17, 2014.
  • Copyright ©ERS 2015.

ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

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Long-term experience in the treatment of α1-antitrypsin deficiency: 25 years of augmentation therapy
Helmut Teschler
European Respiratory Review Mar 2015, 24 (135) 46-51; DOI: 10.1183/09059180.10010714

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Long-term experience in the treatment of α1-antitrypsin deficiency: 25 years of augmentation therapy
Helmut Teschler
European Respiratory Review Mar 2015, 24 (135) 46-51; DOI: 10.1183/09059180.10010714
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    • Introduction
    • Goals of augmentation therapy with AAT
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