title: 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.
authors.affiliation: Division of Respiratory Medicine, Juntendo University, Faculty of Medicine and Graduate School of Medicine; 2-1-1 Hongo; Bunkyo-Ku; Tokyo, Japan.
authors.forename: Katsutoshi
authors.initials: K
authors.lastname: Ando
authors.affiliation: Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University; Seiryo-machi 4-1, Aoba-ku Sendai, Miyagi, Japan.
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Okada
authors.affiliation: Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University; Seiryo-machi 4-1, Aoba-ku Sendai, Miyagi, Japan.
authors.forename: Miki
authors.initials: M
authors.lastname: Akiba
authors.affiliation: Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University; Seiryo-machi 4-1, Aoba-ku Sendai, Miyagi, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Kondo
authors.affiliation: Department of General Thoracic Surgery, Osaka University Graduate School of Medicine; 2-2 Yamadaoka, Suita, Osaka, Japan.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Kawamura
authors.affiliation: Department of General Thoracic Surgery, Osaka University Graduate School of Medicine; 2-2 Yamadaoka, Suita, Osaka, Japan.
authors.forename: Meinoshin
authors.initials: M
authors.lastname: Okumura
authors.affiliation: Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto, Japan.
authors.forename: Fengshi
authors.initials: F
authors.lastname: Chen
authors.affiliation: Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Date
authors.affiliation: Department of General Thoracic, Breast, and Pediatric Surgery, Fukuoka University School of Medicine; 7-45-1 Nanakuma, Jonan-ku, Fukuoka City, Fukuoka, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Shiraishi
authors.affiliation: Department of General Thoracic, Breast, and Pediatric Surgery, Fukuoka University School of Medicine; 7-45-1 Nanakuma, Jonan-ku, Fukuoka City, Fukuoka, Japan.
authors.forename: Akinori
authors.initials: A
authors.lastname: Iwasaki
authors.affiliation: Division of Surgical Oncology, Department of Translational Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences; 1-7-1 Sakamoto, Nagasaki, Japan.
authors.forename: Naoya
authors.initials: N
authors.lastname: Yamasaki
authors.affiliation: Division of Surgical Oncology, Department of Translational Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences; 1-7-1 Sakamoto, Nagasaki, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nagayasu
authors.affiliation: Department of General Thoracic Surgery, Dokkyo Medical University; 880 Kitakobayashi, Mibu-machi, Shimotsuga-gun, Tochigi, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Chida
authors.affiliation: Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center; 1180, Nagasonecho, Kita-Ku, Sakai, Osaka, Japan.|Respiratory Failure Research Group from the Ministry of Health, Labour and Welfare, Japan, (Office) Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University; Kawahara 54, Shogoin, Sakyo-ku, Kyoto, Japan.
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Inoue
authors.affiliation: Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University; Kawahara 54, Shogoin, Sakyo-ku, Kyoto, Japan.|Respiratory Failure Research Group from the Ministry of Health, Labour and Welfare, Japan, (Office) Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University; Kawahara 54, Shogoin, Sakyo-ku, Kyoto, Japan.
authors.forename: Toyohiro
authors.initials: T
authors.lastname: Hirai
authors.affiliation: Division of Respiratory Medicine, Juntendo University, Faculty of Medicine and Graduate School of Medicine; 2-1-1 Hongo; Bunkyo-Ku; Tokyo, Japan.|Respiratory Failure Research Group from the Ministry of Health, Labour and Welfare, Japan, (Office) Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University; Kawahara 54, Shogoin, Sakyo-ku, Kyoto, Japan.
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Seyama
authors.affiliation: Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University; Kawahara 54, Shogoin, Sakyo-ku, Kyoto, Japan.|Respiratory Failure Research Group from the Ministry of Health, Labour and Welfare, Japan, (Office) Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University; Kawahara 54, Shogoin, Sakyo-ku, Kyoto, Japan.
authors.forename: Michiaki
authors.initials: M
authors.lastname: Mishima
mesh_terms: D000328:Adult; D000900:Anti-Bacterial Agents / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007564:Japan; D008175:Lung Neoplasms / Q000601:surgery*; D016040:Lung Transplantation; D018192:Lymphangioleiomyomatosis / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D020123:Sirolimus / Q000008:administration & dosage / Q000627:therapeutic use; D015996:Survival Rate; D055815:Young Adult
chemical_list: D000900:Anti-Bacterial Agents; D020123:Sirolimus