Chen Fengshi, Omasa Mitsugu, Kondo Nobuyuki, Fujinaga Takuji, Shoji Tsuyoshi, Sakai Hiroaki, Bando Toru
Department of Thoracic Surgery, Kyoto University, Sakyo-ku, Kyoto, Japan.
Ann Thorac Surg. 2009 Jan;87(1):e6-7. doi: 10.1016/j.athoracsur.2008.07.107.
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.
肺淋巴管平滑肌瘤病是一种罕见疾病，通常会进展为呼吸衰竭。我们遇到一名在移植肺中复发淋巴管平滑肌瘤病的患者。胸部计算机断层扫描显示肺气肿变化进展。该患者在左支气管吻合术远端部位存在亚临床范围的全周性狭窄。我们用西罗莫司治疗该患者三年。胸部计算机断层扫描显示在这三年后期没有加重迹象，而肺功能测试显示使用西罗莫司后有轻微改善。支气管狭窄也几乎完全消失。这是首例报道的用西罗莫司治疗移植后复发性淋巴管平滑肌瘤病的病例。
