TY - JOUR T1 - Long-term experience in the treatment of α<sub>1</sub>-antitrypsin deficiency: 25 years of augmentation therapy JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 46 LP - 51 DO - 10.1183/09059180.10010714 VL - 24 IS - 135 AU - Helmut Teschler Y1 - 2015/03/01 UR - http://err.ersjournals.com/content/24/135/46.abstract N2 - 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. Overview of long-term experience gained with AAT augmentation therapy for the treatment of AATD http://ow.ly/HmJLR ER -