{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T15:37:30Z","timestamp":1770824250837,"version":"3.50.1"},"reference-count":27,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,11,28]],"date-time":"2019-11-28T00:00:00Z","timestamp":1574899200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Respiratory J"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Lung transplantation (LT) is a reliable therapeutic option for end\u2010stage pulmonary lymphangioleiomyomatosis (LAM). Long\u2010term outcome of LAM recipients after LT remains unknown. The aim of this study was to describe the outcomes of LT for LAM with a long\u2010term follow\u2010up, comparing those for other diseases in the same period.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively reviewed consecutive 145 LT recipients between 1998 and 2015 at Okayama University Hospital with minimum 3\u2010year follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twelve LAM recipients including nine sporadic\u2010LAM and three tuberous sclerosis complex \u2010LAM were identified. Nine of 12 underwent bilateral LT including four living\u2010donor lobar LT. There was no significant difference in overall survival between the two groups. (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.15). Chronic lung allograft dysfunction free survival rate in LAM compared with other diseases tended to be better (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.058). However, the rate of requiring hemodialysis was significantly higher in LAM recipients than in the recipients of other diseases (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.047). Notably, 8 of 12 (67%) LAM patients encountered LAM\u2010related complication including chylothorax and pneumothorax, seven (58%) had proliferative diseases consisting of renal angiomyolipoma and recurrent LAM. Nine patients required mTOR inhibitors for LAM\u2010related problems, contributing to improved control of LAM\u2010related problems. While all nine recipients of bilateral LT have still survived, two patients died of diseases in their native lungs and one required re\u2010LT among three recipients of single LT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Although the rates of LAM\u2010related complications were unexpectedly high in the long term, LT is a feasible therapeutic option for patients with advanced pulmonary LAM.<\/jats:p><\/jats:sec>","DOI":"10.1111\/crj.13108","type":"journal-article","created":{"date-parts":[[2019,11,15]],"date-time":"2019-11-15T13:24:18Z","timestamp":1573824258000},"page":"116-123","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Favorable survival even with high disease\u2010specific complication rates in lymphangioleiomyomatosis after lung transplantation\u2014long\u2010term follow\u2010up of a Japanese 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