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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.

SUBMITTER: Ando K

PROVIDER: S-EPMC4714890 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature

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Lung Transplantation for Lymphangioleiomyomatosis in Japan.

Ando Katsutoshi K Okada Yoshinori Y Akiba Miki M Kondo Takashi T Kawamura Tomohiro T Okumura Meinoshin M Chen Fengshi F Date Hiroshi H Shiraishi Takeshi T Iwasaki Akinori A Yamasaki Naoya N Nagayasu Takeshi T Chida Masayuki M Inoue Yoshikazu Y Hirai Toyohiro T Seyama Kuniaki K Mishima Michiaki M

PloS one 20160115 1

<h4>Background</h4>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.<h4>Methods</h4>In this national survey, we analyzed data from 98 LAM patients registered for lung transplantation in the Japan Organ Transplantation Network.<h4>Results</h4>Transplantation was performed in 57 ...[more]

PMID: 26771878

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Project description:BackgroundLymphangioleiomyomatosis (LAM) is a rare lung disease found primarily in women of childbearing age, characterized by the formation of air-filled cysts, which may be associated with reductions in lung function. An experimental, regional ultra-high resolution CT scan identified an additional volume of cysts relative to standard chest CT imaging, which consisted primarily of ultra-small cysts.Research questionWhat is the impact of these ultra-small cysts on the pulmonary function of patients with LAM?Study design and methodsA group of 103 patients with LAM received pulmonary function tests and a CT examination in the same visit. Cyst score, the percentage lung volume occupied by cysts, was measured by using commercial software approved by the US Food and Drug Administration. The association between cyst scores and pulmonary function tests of diffusing capacity of the lungs for carbon monoxide (Dlco) (% predicted), FEV1 (% predicted), and FEV1/FVC (% predicted) was assessed with statistical analysis adjusted for demographic variables. The distributions of average cyst size and ultra-small cyst fraction among the patients were evaluated.ResultsThe additional cyst volume identified by the experimental, higher resolution scan consisted of cysts of 2.2 ± 0.8 mm diameter on average and are thus labeled the "ultra-small cyst fraction." It accounted for 27.9 ± 19.0% of the total cyst volume among the patients. The resulting adjusted, whole-lung cyst scores better explained the variance of Dlco (P < .001 adjusted for multiple comparisons) but not FEV1 and FEV1/FVC (P = 1.00). The ultra-small cyst fraction contributed to the reduction in Dlco (P < .001) but not to FEV1 and FEV1/FVC (P = .760 and .575, respectively). The ultra-small cyst fraction and average cyst size were correlated with cyst burden, FEV1, and FEV1/FVC but less with Dlco.InterpretationThe ultra-small cysts primarily contributed to the reduction in Dlco, with minimal effects on FEV1 and FEV1/FVC. Patients with lower cyst burden and better FEV1 and FEV1/FVC tended to have smaller average cyst size and higher ultra-small cyst fraction.Clinical trial registrationClinicalTrials.gov; No.: NCT00001465; URL: www.clinicaltrials.gov.

Dataset Information

Lung Transplantation for Lymphangioleiomyomatosis in Japan.

Publications

Lung Transplantation for Lymphangioleiomyomatosis in Japan.

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