title: Sirolimus amelioration of clinical symptoms of recurrent lymphangioleiomyomatosis after living-donor lobar lung transplantation.
abstract: In this study we report the case of a 28-year-old female patient with recurrent lymphangioleiomyomatosis (LAM) in the allografts after bilateral living-donor lobar lung transplantation. Although her post-operative course under immunosuppression with tacrolimus and prednisolone had been uneventful without rejection episodes, she had developed shortness of breath and a progressive chylous effusion with diffuse cystic changes in both lungs 5 years after transplantation. In spite of a diagnosis of having a recurrence of LAM based on radiologic findings and deteriorating pulmonary function, her clinical symptoms, which included dyspnea and chylothorax, were significantly improved after treatment with sirolimus. Although a beneficial effect of sirolimus in the treatment of LAM has not been definitively determined, this report may provide useful information for management of recurrent LAM after lung transplantation.
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: Ryujiro
authors.initials: R
authors.lastname: Sugimoto
authors.forename: Atsunori
authors.initials: A
authors.lastname: Nakao
authors.forename: Masaomi
authors.initials: M
authors.lastname: Yamane
authors.forename: Shinichi
authors.initials: S
authors.lastname: Toyooka
authors.forename: Megumi
authors.initials: M
authors.lastname: Okazaki
authors.forename: Motoi
authors.initials: M
authors.lastname: Aoe
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Seyama
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Date
authors.forename: Takahiro
authors.initials: T
authors.lastname: Oto
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Sano
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D019520:Living Donors; D008168:Lung / Q000000981:diagnostic imaging; D016040:Lung Transplantation; D018192:Lymphangioleiomyomatosis / Q000188:drug therapy*; D012008:Recurrence; D020123:Sirolimus / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D020123:Sirolimus