Durability of Sirolimus for Lymphangioleiomyomatosis

Lymphangioleiomyomatosis: diagnostic imaging in an uncommon post‐menopausal presentation

“…Serum vascular endothelial growth factor D is a non‐invasive biomarker that can enhance the definitive diagnosis . Treatment with sirolimus, a mechanistically effective mammalian target of rapamycin inhibitor, results in significant and sustained improvements in lung function, reduced symptoms, decreased size of angiomyolipomas and resolution of chylous effusions . Lung transplantation is indicated in patients with resistant lymphangioleiomyomatosis and remains the only validated treatment …”