Outcomes and survival in alpha1-antitrypsin deficiency post lung transplantation

Abstract

Alpha1-antitrypsin deficiency (AATD) disease is associated with several inflammatory conditions due to unprotected proteolytic activity of neutrophil elastase and proteinase 3. We observed that patients with ATTD develop early complications post-transplant.

The aim of the study was to identify potential differences in outcomes between AATD and emphysema without AATD (E) following lung transplant.

We retrospectively reviewed the data of 41 patients (27 E and 14 AATD) transplanted between 2005 and 2017. Data collection includes functional baseline of recipients and complications as primary outcome, and secondary end points of survival.

The majority (79%) of AATD patients received double lung transplant. Main complications in AATD cohort included 3 bowel ischemia and perforation, 1 liver cirrhosis and 4 anastomotic complications causing death in 2 patients. Comparison rate of FEV1 decline in AATD and E group in year 1, 5 and 10 showed no statistical difference (P=0.18; P=0.67), same as FEV1 pre and post-transplant between groups. Mortality was higher in AATD (36%) compare to E (30%). Survival curve showed no difference between both groups with median survival AATD 10.8 years (95% CI 0.54 to 5.336) and E 6.4 years (95% CI 0.19 to 1.86, P=0.64).

AATD transplant recipients are predisposed to complications related to their primary underlying disease when compare to E group. However, FEV1 decline had similar trends in both groups with no difference in overall survival.