Articles

SUCCESSFUL TREATMENT OF PULMONARY AND LYMPHATIC MANIFESTATIONS OF LYMPHANGIOLEIOMYOMATOSIS WITH SIROLIMUS

Authors
  • A Hecimovic
  • M Jakopovic
  • G Pavlisa
  • M Jankovic
  • A Vukic-Dugac
  • G Redzepi
  • L Brcic
  • M Samarzija
  • N Gupta

Abstract

Lymphangioleiomyomatosis (LAM) is a rare, progressive, diffuse cystic lung disease predominantly affecting women of childbearing age. Recently treatment with sirolimus was shown to stabilize lung function decline and improve quality of life in patients with LAM. We treated three pre-menopausal women suffering from LAM manifesting as diffuse cystic lung disease, chylous effusions, and lymphangioleioyomas with sirolimus (1-3 mg a day; sirolimus trough levels 2.9-8.5 ng/ml). All three patients had a remarkable response to sirolimus, with resolution of effusions, improvement in lung function and shrinking of abdominal lymphangioleiomyomas. Our case series further complements the literature in that sirolimus is a safe and effective treatment for LAM and its lymphatic manifestations.

Keywords: lymphangioleimyomatosis, lymphangioleiomyoma, chylous ascites, clylothorax, pleural effusion, sirolimus, MILES trial

How to Cite:

Hecimovic, A., Jakopovic, M., Pavlisa, G., Jankovic, M., Vukic-Dugac, A., Redzepi, G., Brcic, L., Samarzija, M. & Gupta, N., (2015) “SUCCESSFUL TREATMENT OF PULMONARY AND LYMPHATIC MANIFESTATIONS OF LYMPHANGIOLEIOMYOMATOSIS WITH SIROLIMUS”, Lymphology 48(2), 97-102.

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Published on
27 Jan 2015
Peer Reviewed