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Zaki KS, Aryan Z, Mehta AC, et al. Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation. World J Transplant. 2016;6(1):249-54doi: 10.5500/wjt.v6.i1.249.
Zaki, K. S., Aryan, Z., Mehta, A. C., Akindipe, O., & Budev, M. (2016). Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation. World journal of transplantation, 6(1), 249-54. https://doi.org/10.5500/wjt.v6.i1.249
Zaki, Khawaja S, et al. "Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation." World journal of transplantation vol. 6,1 (2016): 249-54. doi: https://doi.org/10.5500/wjt.v6.i1.249
Zaki KS, Aryan Z, Mehta AC, Akindipe O, Budev M. Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation. World J Transplant. 2016 Mar 24;6(1):249-54. doi: 10.5500/wjt.v6.i1.249. PMID: 27011924; PMCID: PMC4801802.
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World J Transplant. 2016 Mar 24;6(1):249-54. doi: 10.5500/wjt.v6.i1.249.

Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation.

World journal of transplantation

Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev

Affiliations

  1. Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev, Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, United States.

PMID: 27011924 PMCID: PMC4801802 DOI: 10.5500/wjt.v6.i1.249

Abstract

Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive lethal lung disease primary afflicting young women. LAM is characterized by proliferation of abnormal smooth muscle cells that target the lungs, causing cystic destruction and eventual respiratory failure leading to death. Recent ten year mortality due to end stage LAM has been reported to be approximately 10%-20%, but may vary. The decline in lung function in LAM is gradual, occurring at a rate of about 3% to 15% per year but can vary from patient to patient. But recently therapy with mammalian target of rapamycin (mTOR) inhibitors such as sirolimus has shown promising results in the stabilization of lung function and reduction of chylous effusions in LAM. Lung transplantation is a viable option for patients who continue to have decline in lung function despite mTOR therapy. Unique issues that may occur post-transplant in a recipient with LAM include development of chylous effusion and a risk of recurrence. We describe a case of LAM recurrence in a bilateral lung transplant recipient who developed histological findings of LAM nine years after transplantation.

Keywords: Lung rejection; Lung transplantation; Lymphangioleiomyomatosis; Mammalian target of rapamycin inhibitors; Sirolimus

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