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Effects of Alpha-1 Proteinase Replacement Continuation Post-Lung Transplantation in Alpha-1 Antitrypsin Deficiency: A Single Center Case Series

M. Bremer, A. Feist, M. Mariski, G. Yung, T. Floreth, E. Schonhoft, G. Schooler, E. Golts, K. Afshar

Lung Transplant, University of California San Diego, La Jolla, CA

Meeting: 2020 American Transplant Congress

Abstract number: B-287

Keywords: Graft function, Lung transplantation, N/A, Obilterative bronchiolitis

Session Information

Session Name: Poster Session B: Lung: All Topics

Session Type: Poster Session

Date: Saturday, May 30, 2020

Session Time: 3:15pm-4:00pm

 Presentation Time: 3:30pm-4:00pm

Location: Virtual

*Purpose: Alpha-1 antitrypsin deficiency (AATD) accounts for approximately 6% of all lung transplants (LTx) performed annually. The benefit of replacement with alpha-1 proteinase inhibitor (A1-PI) has not been well studied post-LTx. Our primary objective was to assess benefits in administering A1-PI post-lung transplantation for AATD LTx recipients.

*Methods: A retrospective case series was performed on patients who received LTx for AATD between 2002 and 2018. Data reviewed included AATD phenotype, pulmonary function tests, date of initiation of A1-PI, antibiotic data, photopheresis treatments, and available surveillance bronchoscopies. Endpoints included the change of forced expiratory volume in one second (FEV1) post-transplant, infective episodes, bronchiolitis obliterans syndrome (BOS) staging, and acute lung rejection episodes.

*Results: Out of the 13 AATD LTx recipients, 6 continually received A1-PI beginning prior to transplant (Group 1), and 7 historical control patients were re-introduced to Α1-PI a number of years after LTx (Group 2). All patients received Α1-PI once weekly at a dose of 60 mg/kg. Group 1 experienced a mean FEV1% predicted decline after two years of 6.1% (range: 0.0%-21.0%), and Group 2 experienced a mean decline of 24.2% (range: 13.5%-63.5%). No significant differences noted in frequency of infective episodes between Groups 1 and 2. Only one patient in Group 1 developed BOS about 2.5 years post-transplant, whereas all Group 2 patients developed BOS at a mean of 5.4 years post-transplant (range: 0.7-8.0 years). No patients in Group 1 experienced any acute lung rejection episodes noted from surveillance bronchoscopies, corresponding data not available for Group 2 patients. Figure. Group 1 (blue) and Group 2 (green) FEV1% predicted over time following the baseline FEV1% predicted value for each AATD LTx patient.

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*Conclusions: We report that the continual use of Α1-PI in AATD LTx recipients can result in better maintenance and stabilization of lung functions and potentially less acute cellular rejection episodes early post-LTx. Prospective studies should be performed to confirm a benefit in this population.

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To cite this abstract in AMA style:

Bremer M, Feist A, Mariski M, Yung G, Floreth T, Schonhoft E, Schooler G, Golts E, Afshar K. Effects of Alpha-1 Proteinase Replacement Continuation Post-Lung Transplantation in Alpha-1 Antitrypsin Deficiency: A Single Center Case Series [abstract]. Am J Transplant. 2020; 20 (suppl 3). https://atcmeetingabstracts.com/abstract/effects-of-alpha-1-proteinase-replacement-continuation-post-lung-transplantation-in-alpha-1-antitrypsin-deficiency-a-single-center-case-series/. Accessed May 11, 2025.

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