title: Sirolimus treatment for recurrent lymphangioleiomyomatosis after lung transplantation.
abstract: 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.
authors.affiliation: Department of Thoracic Surgery, Kyoto University, Sakyo-ku, Kyoto, Japan.
authors.forename: Fengshi
authors.initials: F
authors.lastname: Chen
authors.forename: Mitsugu
authors.initials: M
authors.lastname: Omasa
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Kondo
authors.forename: Takuji
authors.initials: T
authors.lastname: Fujinaga
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Shoji
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Sakai
authors.forename: Toru
authors.initials: T
authors.lastname: Bando
mesh_terms: D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D019520:Living Donors; D008134:Long-Term Care; D008175:Lung Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D016040:Lung Transplantation / Q000009:adverse effects* / Q000379:methods; D018192:Lymphangioleiomyomatosis / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D011183:Postoperative Complications / Q000175:diagnosis / Q000188:drug therapy; D012008:Recurrence; D012129:Respiratory Function Tests; D020123:Sirolimus / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D020123:Sirolimus