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Ann Thorac Surg 2009;87:e6-e7. doi:10.1016/j.athoracsur.2008.07.107
© 2009 The Society of Thoracic Surgeons
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Tsuyoshi Shoji
Toru Bando
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Right arrow Lung - transplantation

Case Reports

Sirolimus Treatment for Recurrent Lymphangioleiomyomatosis After Lung Transplantation

Fengshi Chen, MD, Mitsugu Omasa, MD, Nobuyuki Kondo, MD, Takuji Fujinaga, MD, Tsuyoshi Shoji, MD, Hiroaki Sakai, MD, Toru Bando, MD*

Department of Thoracic Surgery, Kyoto University, Sakyo-ku, Kyoto, Japan

Accepted for publication July 29, 2008.

* Address correspondence to Dr Bando, Department of Thoracic Surgery, Kyoto University, 54 Shogoin-Kawahara-cho, Sakyo-ku, Kyoto, 606-8507, Japan (Email: bando{at}kuhp.kyoto-u.ac.jp).

Pulmonary lymphangioleiomyomatosis is a rare disease that generally progresses to respiratory failure. We experienced a patient who had recurring lymphangioleiomyomatosis in the transplanted lungs. A chest computed tomographic scan showed a progressing emphysematous change. The patient had a subclinical extent of pan-circumferential stricture at the distal site of the left bronchial anastomosis. We treated the patient with sirolimus for three years. Chest computed tomography showed no sign of exacerbation during the late 3 years, whereas pulmonary function test revealed a slight increase after the use of sirolimus. Bronchial stricture also disappeared almost completely. This is the first reported case with sirolimus treatment for post-transplant recurrent lymphangioleiomyomatosis.






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