A survey of use of mTOR inhibitors in patients with lymphangioleiomyomatosis listed for lung transplant. (April 2022)

• Record Type: Journal Article
• Authors: Warrior, Krishnan; Leard, Lorriana E.; Nair, Arun R.; Gries, Cynthia J.; Fisher, Andrew J.; Johnson, Simon R.; McCormack, Francis X.; Dilling, Daniel F.
• Is Part Of: Respiratory medicine. Volume 195(2022)
• Publication Date: 2022-04
• Subjects: Lymphangioleiomyomatosis -- Lung transplant -- mTOR inhibitor; Chest -- Diseases -- Periodicals; Chest -- Diseases -- Great Britain -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Appareil respiratoire -- Maladies -- Périodiques; Thorax -- Maladies -- Périodiques; Appareil respiratoire -- Maladies -- Traitement -- Périodiques; Electronic journals; 616.2
• DOI: 10.1016/j.rmed.2022.106779
• Languages: English
• ISSNs: 0954-6111

Abstract

Abstract: Background: Lymphangioleiomyomatosis (LAM) is an uncommon indication for lung transplantation. The use of mechanistic target of rapamycin (mTOR) inhibitors, which are the mainstay of treatment in progressive LAM, in patients awaiting lung transplant is controversial. We sought to examine worldwide practice patterns in use of mTOR inhibitors in LAM patients on the lung transplant waiting list. Methods: We designed and disseminated an online survey about institution-specific practice patterns, particularly regarding listing LAM patients for lung transplant and use of mTOR inhibitors in those patients on the transplant waitlist. Results: Of the 49 unique respondent programs, 83.6% had previously listed a LAM patient for lung transplant. Thirteen centers allowed patients to continue on mTOR inhibitor until time of lung transplant. None of those centers reported any complications or deaths attributable to mTOR inhibitor adverse effects. Conclusion: There exists significant variability in practice patterns concerning the use of mTOR inhibitors in LAM patients on the lung transplant waiting list. Our survey suggests favorable outcomes for those patients that did continue mTOR inhibitor up to time of transplant. Further data regarding the risk of anastomotic complication with use of mTOR inhibitors in the pre-transplant period would help provide clarity in this debate. Highlights: Lymphangioleiomyomatosis is a rare indication for lung transplantation. Use of mTOR inhibitors in LAM patients on the waiting list is controversial. Our survey results suggest excellent outcomes in patients using mTOR inhibitor while on the waitlist. Guidelines regarding use of mTOR inhibitors in LAM patients on the waitlist should be reconsidered.