Durability of Sirolimus for Lymphangioleiomyomatosis

Alexandra Martirossian, Shiwan Shah, Lola Carrete, Jose Valle, Vincent Valentine

Research output: Contribution to journalArticle

1 Scopus citations

Abstract

Lymphangioleiomyomatosis (LAM), a rare, multisystem disorder primarily affecting women of reproductive age, is characterized by cystic-appearing lung lesions, progressive loss of lung function, chylous effusions and renal angiomyolipomas. Sirolimus, an mammalian target of rapamycin inhibitor, has been shown to stabilize lung function, reduce symptoms and resolve chylous effusions in the short term for patients with LAM. Herein, we report a premenopausal female with LAM who experienced complete and durable resolution of her chylothoraces with significant and sustained improvement in lung function on sirolimus.

Original languageEnglish (US)
Pages (from-to)603-607
Number of pages5
JournalThe American journal of the medical sciences
Volume354
Issue number6
DOIs
StatePublished - Dec 1 2017

Keywords

  • Chylothorax
  • Lymphangioleiomyomatosis
  • Sirolimus

ASJC Scopus subject areas

  • Medicine(all)

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