Open AccessFavorable survival even with high disease‐specific complication rates in lymphangioleiomyomatosis after lung transplantation—long‐term follow‐up of a Japanese center
Author(s) -
Kurosaki Takeshi,
Otani Shinji,
Miyoshi Kentaroh,
Okazaki Mikio,
Sugimoto Seiichiro,
Suno Manabu,
Yamane Masaomi,
Kobayashi Motomu,
Oto Takahiro,
Toyooka Shinichi
Publication year - 2020
Publication title -
the clinical respiratory journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.789
H-Index - 33
eISSN - 1752-699X
pISSN - 1752-6981
DOI - 10.1111/crj.13108
Subject(s) - medicine , lymphangioleiomyomatosis , lung transplantation , complication , lung , transplantation , single center , disease , center (category theory) , term (time) , interstitial lung disease , lung disease , overall survival , surgery , pediatrics , chemistry , physics , quantum mechanics , crystallography
Background Lung transplantation (LT) is a reliable therapeutic option for end‐stage pulmonary lymphangioleiomyomatosis (LAM). Long‐term 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‐term follow‐up, comparing those for other diseases in the same period. Methods We retrospectively reviewed consecutive 145 LT recipients between 1998 and 2015 at Okayama University Hospital with minimum 3‐year follow‐up. Results Twelve LAM recipients including nine sporadic‐LAM and three tuberous sclerosis complex ‐LAM were identified. Nine of 12 underwent bilateral LT including four living‐donor lobar LT. There was no significant difference in overall survival between the two groups. ( P = 0.15). Chronic lung allograft dysfunction free survival rate in LAM compared with other diseases tended to be better ( P = 0.058). However, the rate of requiring hemodialysis was significantly higher in LAM recipients than in the recipients of other diseases ( P = 0.047). Notably, 8 of 12 (67%) LAM patients encountered LAM‐related complication including chylothorax and pneumothorax, seven (58%) had proliferative diseases consisting of renal angiomyolipoma and recurrent LAM. Nine patients required mTOR inhibitors for LAM‐related problems, contributing to improved control of LAM‐related problems. While all nine recipients of bilateral LT have still survived, two patients died of diseases in their native lungs and one required re‐LT among three recipients of single LT. Conclusion Although the rates of LAM‐related complications were unexpectedly high in the long term, LT is a feasible therapeutic option for patients with advanced pulmonary LAM.