Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation?
Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation?
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DOI：
10.1586/17476348.2014.956728
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发表时间：
2014-11
影响因子：
3.9
通讯作者：
S. El-Chemaly;H. Goldberg;A. Glanville
中科院分区：
文献类型：
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作者：
S. El-Chemaly;H. Goldberg;A. Glanville
淋巴管平滑肌瘤病（LAM）是一种罕见的囊性肺病，其特征是结节性硬化症基因（TSC 2）突变的平滑肌样细胞（LAM细胞）增殖，导致哺乳动物雷帕霉素靶蛋白（mTOR）激活。雷帕霉素是mTOR通路的抑制剂，在一项具有里程碑意义的临床试验中显示，只要持续使用，它就能阻止肺功能的下降。患有严重肺部LAM的女性仍然需要进行肺移植。移植后立即使用mTOR通路抑制剂与支气管吻合口裂开有关，这是肺移植的潜在致命并发症。目前，建议LAM患者一旦被列为肺移植，就停止服用雷帕霉素，这可能会导致在等待器官移植时肺功能更快下降。在这里，我们回顾了现有的证据，并讨论了潜在的建议，为管理的mTOR通路的抑制剂，而等待肺移植。
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 (TSC2), 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.