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Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation?
Open accessAbstract
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.
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Everolimus has a shorter half-life than sirolimus.
“Given its shorter half-life, everolimus is usually preferred to sirolimus for listed patients.(316)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [316]
Disease recurrence occurs after lung transplant for lymphangioleiomyomatosis but does not appear to limit survival.
“While disease recurrence does occur post-transplant, it does not appear to limit survival.(303, 304, 309, 311, 316–318)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [316]
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- Glanville AR, 2014, [Epub ahead of print]
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Metadata
- Authors
- El-Chemaly, S., Goldberg, H. J., & Glanville, A. R.
- Affiliations
- Brigham and Women's Hospital (US)
- St Vincent's Hospital Sydney (AU)
- Venue
- Expert Review of Respiratory Medicine
- Publisher
- Informa UK Limited
- Published
- 2014-09-09
- Volume
- 8
- Issue
- 6
- Pages
- 657-660
- ISSN
- 1747-6348 (ISSN-L)
- 1747-6356
- OpenAlex
- W2136681516
- Semantic Scholar
- 73ee0f7de5762198a54c8f8a888020293be7563b
- Cited by
- 25
- References
- 17
- Citations by year
- 2025: 3 · 2024: 1 · 2023: 3 · 2022: 3 · 2021: 5 · 2020: 2 · 2019: 3 · 2018: 2 · 2016: 1 · 2015: 2
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Expert Review of Respiratory Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Tuberous Sclerosis Complex Research
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Medical Imaging and Pathology Studies
- Keywords
- lymphangioleiomyomatosis, mTOR inhibitors, rapamycin, lung transplantation, pre-transplant management, TSC2 mutation, lung function decline, women
- MeSH terms
- Female, Humans, Lung Neoplasms, Lung Transplantation, Lymphangioleiomyomatosis, Sirolimus, TOR Serine-Threonine Kinases
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