Lung Transplantation for Lymphangioleiomyomatosis in Japan

PloS One
Katsutoshi AndoRespiratory Failure Research Group of the Japanese Ministry of Health, Labour, and Welfare

Abstract

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. In this national survey, we analyzed data from 98 LAM patients registered for lung transplantation in the Japan Organ Transplantation Network. 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). Lung transplantation is a satisfactory therapeutic option for advanced LAM, but the circumstances for pre-transplantation LAM patients are likely to a...Continue Reading

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Citations

Nov 11, 2017·Journal of the Intensive Care Society·Emma Jackson, Raj Nichani
Nov 24, 2017·Respiration; International Review of Thoracic Diseases·Cécile DaccordRomain Lazor
Jun 22, 2018·Current Opinion in Pulmonary Medicine·Sergio HarariVincent Cottin
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Jul 16, 2019·The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation·Muhammad Umair KhawarNishant Gupta
Dec 12, 2020·Breathe·Anne M O'MahonyCormac McCarthy
Aug 6, 2021·European Respiratory Review : an Official Journal of the European Respiratory Society·Yasmine RebaineVincent Cottin
Aug 28, 2021·International Journal of Molecular Sciences·Yen-Lin HuangHsao-Hsun Hsu
Aug 23, 2021·The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation·Lorriana E LeardKathleen J Ramos
Aug 31, 2021·The Lancet. Respiratory Medicine·Cormac McCarthyFrancis X McCormack

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Methods Mentioned

BETA
biopsy

Software Mentioned

SPSS

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