TY - JOUR T1 - The withdrawal of replacement therapy and outcomes in Alpha1-antitrypsin deficiency lung transplant recipients JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00055-2019 SP - 1900055 AU - J. Kleinerova AU - P. Ging AU - C. Rutherford AU - I. Lawrie AU - S. Winward AU - D. Eaton AU - K.C. Redmond AU - J.J. Egan Y1 - 2019/01/01 UR - http://erj.ersjournals.com/content/early/2019/02/13/13993003.00055-2019.abstract N2 - Alpha-1-antitrypsin deficiency (AATD) accounts for 5% of lung transplant performed worldwide. Ireland has a high frequency of 1 in 25 for the Z allele and 1 in 10 for the S allele [1]. These variants are associated with decreased production of alpha-1 antitrypsin (AAT), which predisposes to unprotected proteolytic activity of neutrophil elastase (NE) and proteinase 3 (PR3) linked to an increased risk of emphysema and hepatic disease. Associated inflammatory conditions such as vasculitis, panniculitis and inflammatory bowel disease are also suspected to be caused by uncontrolled neutrophil proteinases activity [2–4]. Vascular problems including aortic aneurysms in AATD patients are more severe and may reflect abnormal elastic tissue properties potentially impacting on wound healing [5].FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Kleinerova has nothing to disclose.Conflict of interest: Prof. Ging has nothing to disclose.Conflict of interest: Dr. Rutherford has nothing to disclose.Conflict of interest: Dr. Lawrie has nothing to disclose.Conflict of interest: Ms. Winward has nothing to disclose.Conflict of interest: Ms. Eaton has nothing to disclose.Conflict of interest: Prof. Redmond has nothing to disclose.Conflict of interest: Prof. Egan has nothing to disclose. ER -