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Lung India Official publication of Indian Chest Society  
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CASE REPORT
Year : 2019  |  Volume : 36  |  Issue : 4  |  Page : 349-352

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas


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

Correspondence Address:
Dr. Kamonpun Ussavarungsi
Division of Pulmonary and Critical Care, Department of Internal Medicine, University of Iowa, 200 Hawkins Drive, C33 GH Iowa City, IA 52242
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/lungindia.lungindia_433_18

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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.


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