Continuation of mTOR Inhibition in LAM Patients Listed for Lung Transplant is Safe

Autor: Reda E. Girgis, Daniel C. Chambers, Souheil El-Chemaly, Vincent G. Valentine, Daniel F. Dilling, D.R. Darley, K. Warrior, Allan R. Glanville, Mark Holmes
Rok vydání: 2020
Předmět:
Zdroj: The Journal of Heart and Lung Transplantation. 39:S324
ISSN: 1053-2498
DOI: 10.1016/j.healun.2020.01.334
Popis: Purpose Use of mammalian target of rapamycin (mTOR) inhibitors in lymphangioleiomyomatosis (LAM) is now standard of care.1 However, in LAM patients listed for lung transplant (LTX), mTOR inhibitors are often held, as sirolimus has been associated with anastomotic dehiscence2,3. This hold can lead to accelerated respiratory failure. In a survey of LTX programs, 35.4% did not allow patients to remain on mTOR therapy while on the wait list4. We sought to collect a case series of patients who remained on mTOR inhibitors while on the waitlist and describe their outcomes after LTX. Methods Pulmonologists who had managed LAM patients with LTX and continued mTOR inhibitor were contacted. Information was collected from those providers, pertaining to mTOR inhibitor use, airway complications, and transplant outcomes. Results Eight LAM patients were identified as having been on mTOR inhibitor up until call-in for LTX. Average ages at diagnosis and at LTX were 38.5 years and 48.2 years, respectively. The mTOR inhibitor was started 46.8 months before LTX, with a mean time between listing and LTX of 10.5 months. Seven patients had sporadic LAM, and one had tuberous sclerosis complex-associated LAM. mTOR inhibitor was continued at time of listing in all patients. Three were on sirolimus prior to listing; in two of those patients, sirolimus was switched to everolimus at time of listing. After LTX, mTOR inhibition was resumed in 4 of 8 patients, at a mean of 0.85 years after LTX. One patient had a significant airway complication, a partial right mainstem dehiscence with subsequent stenosis requiring stenting, and recurrent bronchopulmonary infections. She remains alive at 11.3 months after transplant at time of survey. No other patients had wound healing issues. All were alive at time of survey, at a median of 34.9 months after LTX (range, 11.3 to 115.0 months). Conclusion While controversy abounds regarding the use of mTOR inhibitors during while listed for LTX in LAM patients, this case series suggest that continuation of mTOR inhibitor during waitlist phase before LTX is reasonable and safe. Programs should consider allowance of mTOR inhibition in LAM patients on the LTX waitlist. References: 1. McCormack FX et al. N Engl J Med. 2011;364(17):1595 2. King-Biggs MB et al. Transplantation 2003;75:1437 3. Groetzner J et al. J Heart Lung Transplant 2004;23:632 4. Dilling, DF et al. J Heart Lung Transplant. 2018 37(4), S457
Databáze: OpenAIRE