Abstract RATIONALE Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease driven by nodules containing “LAM cells” and recruited LAM-associated fibroblasts. Although rapamycin reduces lung function loss, some patients continue to decline, meaning additional therapies are needed. OBJECTIVES To investigate how the LAM nodule environment affects LAM cell proliferation and the response to rapamycin. METHODS Proteins […]
Extracellular matrix deposition drives disease progression and reduces rapamycin response in lymphangioleiomyomatosis.
May 1, 2026
Eur Respir J