Experience with Alpha-1 Proteinase Replacement Post-Lung Transplantation in Alpha-1 Antitrypsin Deficiency: A Single Center Case Series

Abstract

Alpha-1 antitrypsin deficiency (AATD) accounts for approximately 5% of lung transplants (LTx) performed annually. No studies have addressed the potential benefit of ongoing alpha-1 proteinase inhibitor (A1-PI) replacement to AATD patients post-LTx. Our primary objective was to assess potential benefits of continually administering A1-PI from pre- to post-transplantation for AATD LTx recipients. A retrospective case series was performed on AATD LTx recipients between 2002 and 2018. Data reviewed included date of A1-PI initiation, pulmonary function tests, and surveillance bronchoscopies. Endpoints included the change of forced expiratory volume in one-second (FEV1), infective episodes, chronic lung allograft dysfunction (CLAD), and acute rejection episodes. Out of the 13 AATD LTx recipients, 6 continually received A1-PI beginning prior to transplant (Group 1), and 7 were re-introduced to Α1-PI a number of years after LTx (Group 2). After two years, Group 1 experienced a median FEV1% predicted decline of 0.0%, and Group 2 experienced a median decline of 15.0%. No differences noted in frequency of infective episodes. One patient in Group 1 developed CLAD about 2.5 years post-LTx, whereas all Group 2 patients developed CLAD at a mean of 5.4 years post-LTx. No Group 1 patients experienced acute lung rejection episodes noted from surveillance bronchoscopies, corresponding data not available for Group 2. We report that the continual use of Α1-PI in AATD LTx recipients is associated with better maintenance and stabilization of lung function and potentially less acute lung rejection episodes early post-LTx. Prospective studies should be performed to confirm possible benefits.