Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation?

[lymphangioleiomyomatosis]

Lymphangioleiomyomatosis (LAM ) is a rare cystic lung disease characterized by proliferation of smooth muscle like cells (LAM cells ) that have mutations in the tuberous sclerosis gene (TSC 2 ), leading to the activation of the mammalian target of rapamycin (mTOR ). Rapamycin , an inhibitor of the mTOR pathway , has been shown in a landmark clinical trial to halt the decline in lung function , as long as it is used continuously . Women with severe pulmonary LAM still progress to require lung transplantation . The use of inhibitors of the mTOR pathway immediately after transplant has been linked to bronchial anastomotic dehiscence , a potentially fatal complication of lung transplantation . Currently , it is recommended that women with LAM stop taking rapamycin once listed for lung transplant , which could potentially lead to faster lung function decline while awaiting organ transplantation . Here we review the existing evidence and discuss potential recommendations for the management of the inhibitors of the mTOR pathway while awaiting lung transplantation .