Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Transplant. Mar 24, 2016; 6(1): 249-254
Published online Mar 24, 2016. doi: 10.5500/wjt.v6.i1.249
Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation
Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev
Khawaja S Zaki, Zahra Aryan, Atul C Mehta, Olufemi Akindipe, Marie Budev, Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, United States
Atul C Mehta, Lerner College of Medicine, Bouncore Family Endowed Chair in Lung Transplantation, Cleveland Clinic, Cleveland, OH 44195, United States
Author contributions: All authors contributed to acquisition of data, writing and revision of the manuscript.
Institutional review board statement: This is a single case report and hence no institutional review board statement is required.
Informed consent statement: Verbal consent obtained from the deceased patient’s daughter.
Conflict-of-interest statement: We have no financial relationship to disclose.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Atul C Mehta, MD, FACP, FCCP, Professor of Medicine, Staff, Lerner College of Medicine, Bouncore Family Endowed Chair in Lung Transplantation, Cleveland Clinic, 2049 E 100th St, Cleveland, OH 44195, United States. mehtaa1@ccf.org
Telephone: +1-216-4442911 Fax: +1-216-4458160
Received: May 9, 2015
Peer-review started: May 11, 2015
First decision: July 26, 2015
Revised: December 1, 2015
Accepted: December 13, 2015
Article in press: December 15, 2015
Published online: March 24, 2016
Processing time: 314 Days and 8.1 Hours
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: Lymphangioleiomyomatosis; Mammalian target of rapamycin inhibitors; Lung transplantation; Sirolimus; Lung rejection

Core tip: Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive lethal lung disease characterized by proliferation of abnormal smooth muscle cells that target the lungs, causing cystic destruction and eventual respiratory failure and death. Mammalian target of rapamycin (mTOR) inhibitors such as sirolimus have shown promise in stabilization of lung function. Lung transplantation is a viable option when lung function continues to decline despite use of mTOR inhibitors. However, recurrence of LAM in transplanted lung has been reported. We describe a case of LAM recurrence in a bilateral lung transplant recipient nine years after transplantation, our therapeutic approach once recurrence was documented with review of the literature.