Sirolimus amelioration of clinical symptoms of recurrent lymphangioleiomyomatosis after living-donor lobar lung transplantation

J Heart Lung Transplant. 2008 Aug;27(8):921-4. doi: 10.1016/j.healun.2008.05.012. Epub 2008 Jun 30.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Living Donors*
  • Lung / diagnostic imaging
  • Lung Transplantation*
  • Lymphangioleiomyomatosis / drug therapy*
  • Recurrence
  • Sirolimus / therapeutic use*
  • Tomography, X-Ray Computed
  • Treatment Outcome


  • Immunosuppressive Agents
  • Sirolimus