Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis.

Abstract

OBJECTIVES:

Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that generally results in progressive decline in lung function. Despite advancement of pharmacological therapy for LAM, lung transplantation remains an important option for women with end-stage LAM.

METHODS:

Patients with LAM undergoing lung transplantation at the Mayo Clinic campuses in Rochester, Minnesota and Jacksonville, Florida since 1995 were retrospectively reviewed.

RESULTS:

Overall, 12 women underwent lung transplantation. Nine of 12 (75%) underwent double lung transplant. The mean age was 42 ± 8 years at the time of transplant. One patient (8%) had a chylothorax and 7 (58%) had recurrent pneumothoraces, 4 (33%) of which required pleurodesis. All had diffuse, cystic lung disease on chest CT consistent with LAM which was confirmed in the explant of all patients. The average length of ICU and hospital stays were 5 ± 4 and 19 ± 19 days, respectively. Mild to moderate anastomotic ischemia was evident in all patients but resolved with time. No patient was treated with sirolimus pre-transplant. Seven patients received sirolimus post-transplant; however, clinical benefit was documented in only 2 patients, 1 of which was treated for large retroperitoneal cysts with ureteral obstruction and another with persistent chylothorax and retroperitoneal lymphangioleimyomas. Five patients are deceased. The median survival by Kaplan-Meier analysis was 119 months with a median follow-up of 68 months (range 2-225 months).

CONCLUSIONS:

Lung transplant remains a viable treatment for patients with end-stage LAM. The role of sirolimus peri-transplantation remains ill-defined.

• Authors: Kamonpun Ussavarungsi, Xiaowen Hu, J P Scott, David B Erasmus, Jorge M Mallea, Francisco Alvarez, Augustine S Lee, Cesar A Keller, Jay H Ryu, Charles D Burger
• Journal: Respiratory medicine (Respir Med) Vol. 109 Issue 10 Pg. 1354-9 (Oct 2015) ISSN: 1532-3064 [Electronic] England
• PMID: 26321137 (Publication Type: Journal Article)
• Copyright: Copyright © 2015 Elsevier Ltd. All rights reserved.

Chemical References

• Immunosuppressive Agents
• Sirolimus

Topics

• Adult
• Echocardiography (methods)
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Lung Neoplasms (diagnostic imaging, pathology, surgery)
• Lung Transplantation (adverse effects, methods)
• Lymphangioleiomyomatosis (diagnostic imaging, pathology, surgery)
• Middle Aged
• Pleurodesis (methods)
• Retrospective Studies
• Severity of Illness Index
• Sirolimus (therapeutic use)
• Tomography, X-Ray Computed (methods)
• Treatment Outcome