Lung Transplantation for Lymphangioleiomyomatosis in Japan.

Abstract

BACKGROUND:

Lung transplantation has been established as the definitive treatment option for patients with advanced lymphangioleiomyomatosis (LAM). However, the prognosis after registration and the circumstances of lung transplantation with sirolimus therapy have never been reported.

METHODS:

In this national survey, we analyzed data from 98 LAM patients registered for lung transplantation in the Japan Organ Transplantation Network.

RESULTS:

Transplantation was performed in 57 patients as of March 2014. Survival rate was 86.7% at 1 year, 82.5% at 3 years, 73.7% at 5 years, and 73.7% at 10 years. Of the 98 patients, 21 had an inactive status and received sirolimus more frequently than those with an active history (67% vs. 5%, p<0.001). Nine of twelve patients who remained inactive as of March 2014 initiated sirolimus before or while on a waiting list, and remained on sirolimus thereafter. Although the statistical analysis showed no statistically significant difference, the survival rate after registration tended to be better for lung transplant recipients than for those who awaited transplantation (p = 0.053).

CONCLUSIONS:

Lung transplantation is a satisfactory therapeutic option for advanced LAM, but the circumstances for pre-transplantation LAM patients are likely to alter with the use of sirolimus.

• Authors: Katsutoshi Ando, Yoshinori Okada, Miki Akiba, Takashi Kondo, Tomohiro Kawamura, Meinoshin Okumura, Fengshi Chen, Hiroshi Date, Takeshi Shiraishi, Akinori Iwasaki, Naoya Yamasaki, Takeshi Nagayasu, Masayuki Chida, Yoshikazu Inoue, Toyohiro Hirai, Kuniaki Seyama, Michiaki Mishima, Respiratory Failure Research Group of the Japanese Ministry of Health, Labour, and Welfare
• Journal: PloS one (PLoS One) Vol. 11 Issue 1 Pg. e0146749 ( 2016) ISSN: 1932-6203 [Electronic] United States
• PMID: 26771878 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Anti-Bacterial Agents
• Sirolimus

Topics

• Adult
• Anti-Bacterial Agents (administration & dosage, therapeutic use)
• Female
• Humans
• Japan
• Lung Neoplasms (surgery)
• Lung Transplantation
• Lymphangioleiomyomatosis (surgery)
• Male
• Middle Aged
• Retrospective Studies
• Sirolimus (administration & dosage, therapeutic use)
• Survival Rate
• Young Adult