Lung Transplantation Outcomes and Peritransplant Sirolimus Use in Lymphangioleiomyomatosis

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 Scienti...

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Main Authors: Emily L. Larson, BS, Reed T. Jenkins, BA, Jessica M. Ruck, MD, Laura B. Zeiser, MS, Alice L. Zhou, MS, Alfred J. Casillan, MD, PhD, Dorry L. Segev, MD, Allan B. Massie, MD, Jinny S. Ha, MD, Pali D. Shah, MD, Christian A. Merlo, MD, MPH, Errol L. Bush, MD
Format: Article
Language:English
Published: Elsevier 2025-03-01
Series:Annals of Thoracic Surgery Short Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2772993124003231
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author Emily L. Larson, BS
Reed T. Jenkins, BA
Jessica M. Ruck, MD
Laura B. Zeiser, MS
Alice L. Zhou, MS
Alfred J. Casillan, MD, PhD
Dorry L. Segev, MD
Allan B. Massie, MD
Jinny S. Ha, MD
Pali D. Shah, MD
Christian A. Merlo, MD, MPH
Errol L. Bush, MD
author_facet Emily L. Larson, BS
Reed T. Jenkins, BA
Jessica M. Ruck, MD
Laura B. Zeiser, MS
Alice L. Zhou, MS
Alfred J. Casillan, MD, PhD
Dorry L. Segev, MD
Allan B. Massie, MD
Jinny S. Ha, MD
Pali D. Shah, MD
Christian A. Merlo, MD, MPH
Errol L. Bush, MD
author_sort Emily L. Larson, BS
collection DOAJ
description 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.
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spelling doaj-art-4d47bb1162924d30b3fd807c2596fa7a2025-08-20T02:11:12ZengElsevierAnnals of Thoracic Surgery Short Reports2772-99312025-03-013113313710.1016/j.atssr.2024.07.011Lung Transplantation Outcomes and Peritransplant Sirolimus Use in LymphangioleiomyomatosisEmily L. Larson, BS0Reed T. Jenkins, BA1Jessica M. Ruck, MD2Laura B. Zeiser, MS3Alice L. Zhou, MS4Alfred J. Casillan, MD, PhD5Dorry L. Segev, MD6Allan B. Massie, MD7Jinny S. Ha, MD8Pali D. Shah, MD9Christian A. Merlo, MD, MPH10Errol L. Bush, MD11Division of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, MarylandDivision of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, MarylandDivision of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, MarylandDivision of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, MarylandDivision of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, MarylandDivision of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, MarylandDepartment of Surgery, NYU Langone School of Medicine, New York, New York; Department of Population Health, NYU Grossman School of Medicine and Langone Health, New York, New York; Scientific Registry of Transplant Recipients, Minneapolis, MinnesotaDepartment of Surgery, NYU Langone School of Medicine, New York, New York; Department of Population Health, NYU Grossman School of Medicine and Langone Health, New York, New YorkDivision of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, MarylandDepartment of Population Health, NYU Grossman School of Medicine and Langone Health, New York, New York; Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins Hospital, Baltimore, MarylandDepartment of Population Health, NYU Grossman School of Medicine and Langone Health, New York, New York; Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins Hospital, Baltimore, MarylandDivision of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, Maryland; Address correspondence to Dr Bush, Division of Thoracic Surgery, Department of Surgery, Johns Hopkins Hospital, 1800 Orleans St, Baltimore, MD, 21287.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.http://www.sciencedirect.com/science/article/pii/S2772993124003231
spellingShingle Emily L. Larson, BS
Reed T. Jenkins, BA
Jessica M. Ruck, MD
Laura B. Zeiser, MS
Alice L. Zhou, MS
Alfred J. Casillan, MD, PhD
Dorry L. Segev, MD
Allan B. Massie, MD
Jinny S. Ha, MD
Pali D. Shah, MD
Christian A. Merlo, MD, MPH
Errol L. Bush, MD
Lung Transplantation Outcomes and Peritransplant Sirolimus Use in Lymphangioleiomyomatosis
Annals of Thoracic Surgery Short Reports
title Lung Transplantation Outcomes and Peritransplant Sirolimus Use in Lymphangioleiomyomatosis
title_full Lung Transplantation Outcomes and Peritransplant Sirolimus Use in Lymphangioleiomyomatosis
title_fullStr Lung Transplantation Outcomes and Peritransplant Sirolimus Use in Lymphangioleiomyomatosis
title_full_unstemmed Lung Transplantation Outcomes and Peritransplant Sirolimus Use in Lymphangioleiomyomatosis
title_short Lung Transplantation Outcomes and Peritransplant Sirolimus Use in Lymphangioleiomyomatosis
title_sort lung transplantation outcomes and peritransplant sirolimus use in lymphangioleiomyomatosis
url http://www.sciencedirect.com/science/article/pii/S2772993124003231
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