Lung Transplantation Outcomes in Lymphangioleiomyomatosis

Abstract

Lymphangioleiomyomatosis (LAM) is a rare slowly progressive disease that leads to respiratory failure. The aim of this study was to analyse the results of lung transplantation (LT) in LAM patients and the sirolimus effect in LT. LAM patients from a referral centre were retrospectively reviewed. Those who underwent LT were considered for the analysis. Demographics, postoperative complications, survival, recurrence of LAM and pulmonary function test (PFT) were analysed. All patients followed a standard triple immunosuppressive therapy based on tacrolimus, mycophenolate mofetil and corticosteroids. Sirolimus was introduced in our centre in November 2007 as a treatment for LAM patients and was indicated in cases of abnormal FEV1 or presence of angiomyolipomas. From 1980 to 2018, 76 LAM patients have been followed-up. Of these, 28 (37%) patients underwent LT. Twenty (71%) were bilateral LT (BLT) and 8 (29%) single LT (SLT). Pre-transplant PFT were: FVC 53±18%, FEV1 35±18% and FEV1% 48±19%. The best post-transplant pulmonary function was achieved at a median of 8 (7-13) months and 18 (8-35) months for SLT and BLT, respectively. Following BLT, the best PFT results were FVC 85±14%, FEV1 88±14% and FEV1% 104±20. In case of SLT, the best FVC was 63 ±12%, FEV1 53±7% and FEV1% 85±10%. Two (7%) patients developed LAM recurrence, in both cases, immunosuppressive treatment was adjusted and the purine synthesis inhibitor was changed to sirolimus. Survival after LT was 67% at 5 years and 58% at 10 years with a mean follow-up after LT of 11.7 years. Since the introduction of sirolimus in 2007, no newly diagnosed LAM cases were submitted to LT. While on sirolimus only 9 patients underwent LT. Survival in LAM patients submitted to LT exceeds 10 years. LT in LAM patients allows an improvement in spirometric values. A significant decrease in the number of LT indications in LAM has been observed since the introduction of sirolimus treatment.