Lung Transplantation Outcomes and Peritransplant Sirolimus Use in Lymphangioleiomyomatosis

Abstract

Background: With the introduction of sirolimus as medical therapy for lymphangioleiomyomatosis (LAM), an updated evaluation of LAM lung transplant (LT) outcomes and characterization of peritransplant sirolimus use is needed. Methods: We identified adult LT recipients from 2005-2021 using the Scientific Registry of Transplant Recipients database and stratified by diagnosis (LAM vs other). Multivariable Cox regression was performed to calculate the adjusted hazard ratio for LAM vs other diagnoses. A pharmacy claims database was linked to provide sirolimus prescription information, and a subgroup analysis comparing outcomes with pre- vs posttransplant sirolimus use was performed. Results: Of 32,337 recipients identified, 156 (0.5%) were diagnosed with LAM. Operative complications, including airway dehiscence, did not significantly differ between groups. After adjusting for donor and recipient characteristics, LAM diagnosis was associated with 45% lower mortality than other diagnoses. Among recipients with pharmacy data, 32% were prescribed sirolimus at any point. Compared with only post-LT use only, recipients with pre-LT sirolimus use had increased mortality (log-rank P = .003). Conclusions: This study supports lung transplant as a treatment for severe pulmonary LAM and identifies increased mortality associated with pre-LT sirolimus, though this may be due to uncharacterized baseline differences.