Sirolimus amelioration of clinical symptoms of recurrent lymphangioleiomyomatosis after living-donor lobar lung transplantation.
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.
Sugimoto
Ryujiro
R
Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
Nakao
Atsunori
A
Yamane
Masaomi
M
Toyooka
Shinichi
S
Okazaki
Megumi
M
Aoe
Motoi
M
Seyama
Kuniaki
K
Date
Hiroshi
H
Oto
Takahiro
T
Sano
Yoshifumi
Y
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
D007166:Immunosuppressive Agents; D020123:Sirolimus