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Lung India. 2019 Jul-Aug;36(4):349-352. doi: 10.4103/lungindia.lungindia_433_18.

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas.

Author information

1
Division of Pulmonary and Critical Care, Department of Internal Medicine, University of Iowa, Iowa City, IA, USA.
2
Division of Cardiovascular and Pulmonary Imaging, Department of Radiology, University of Iowa, Iowa City, IA, USA.
3
Division of Pulmonary and Critical Care, Department of Internal Medicine, Mayo Clinic, Jacksonville, FL, USA.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic lung destruction and abdominal tumors, including lymphangioleiomyomas, which frequently occur in the retroperitoneal region. Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5-15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low-dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM.

KEYWORDS:

Low-dose sirolimus; lymphangioleimyomas; lymphangioleiomyomatosis

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