title: Therapeutic Effect of Sirolimus for Lymphangioleiomyomatosis Remaining in the Abdominopelvic Region After Lung Transplantation: A Case Report.
abstract: OBJECTIVE
  Sirolimus (SRL) is used to treat pulmonary lymphangioleiomyomatosis (P-LAM). There is limited evidence that SRL has systemic efficacy for the patients with extrapulmonary lymphangioleiomyomatosis (E-LAM) remaining after lung transplantation (LT) for P-LAM. This report examines the efficacy of SRL treatment for the patient with E-LAM remaining after an LT for P-LAM.
  
  
  METHODS
  The course of the patient's recovery from an LT for P-LAM was complicated by lymphedema in the left femoral region that was caused by two E-LAM lesions remaining in the left pelvic cavity and in the retroperitoneal area. After the LT was performed, the patient started SRL treatment to reduce the E-LAM lesions. The daily SRL dose, selected based on the standard SRL dose for P-LAM, was initiated at 1 mg/d and was maintained at 2 mg/d. The remaining E-LAM lesions and lymphedema in the left femoral region improved in approximately 9 months after the LT with the administration of both SRL and the standard immunosuppressive therapy used by Okayama University Hospital, including tacrolimus, mycophenolate mofetil, and prednisolone. The SRL and tacrolimus trough concentrations in whole blood were maintained within the therapeutic window for the next 1.5 years after initiation of SRL treatment. The patient experienced no severe adverse events that required discontinuation of the SRL treatment during this time.
  
  
  CONCLUSIONS
  The patients with remaining E-LAM lesions may receive SRL treatment to improve the quality of life after LT for P-LAM as effective therapy in cases where the patient's recovery is complicated by E-LAM lesions.
authors.affiliation: Division of Oncology Pharmaceutical Care & Science, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Ito
authors.affiliation: Division of Oncology Pharmaceutical Care & Science, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan. Electronic address: sunoma@pharm.okayama-u.ac.jp.
authors.forename: M
authors.initials: M
authors.lastname: Suno
authors.affiliation: Division of Oncology Pharmaceutical Care & Science, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Sakamoto
authors.affiliation: Division of Oncology Pharmaceutical Care & Science, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Yoshizaki
authors.affiliation: Division of Oncology Pharmaceutical Care & Science, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Yamamoto
authors.affiliation: Division of Oncology Pharmaceutical Care & Science, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Nakanishi
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Hirano
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Irie
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kurosaki
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Otani
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Yamane
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Sugimoto
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Miyoshi
authors.affiliation: Department of Thoracic Surgery, Okayama University Hospital, Okayama, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Oto
mesh_terms: D000005:Abdomen / Q000473:pathology; D000328:Adult; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008175:Lung Neoplasms / Q000188:drug therapy* / Q000473:pathology; D016040:Lung Transplantation; D018192:Lymphangioleiomyomatosis / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D008209:Lymphedema / Q000188:drug therapy / Q000209:etiology; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D018365:Neoplasm, Residual; D010388:Pelvis / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D011788:Quality of Life; D020123:Sirolimus / Q000627:therapeutic use*; D016559:Tacrolimus / Q000627:therapeutic use
chemical_list: D007166:Immunosuppressive Agents; D011239:Prednisolone; D009173:Mycophenolic Acid; D020123:Sirolimus; D016559:Tacrolimus