Lymphangioleiomyomatosis

Lymphangioleiomyomatosis is a rare, progressive multisystem disease characterized by the abnormal clonal proliferation of atypical smooth muscle-like cells throughout the pulmonary parenchyma, axial lymphatics, and vascular structures Pathophysiologically driven by hyperactivation of the mechanistic target of rapamycin signaling pathway, the condition leads to diffuse cystic lung destruction, recurrent spontaneous pneumothoraces, chylous pleural effusions, progressive dyspnea, and extrapulmonary...

Lymphangioleiomyomatosis is a rare, progressive multisystem disease characterized by the abnormal clonal proliferation of atypical smooth muscle-like cells throughout the pulmonary parenchyma, axial lymphatics, and vascular structures Pathophysiologically driven by hyperactivation of the mechanistic target of rapamycin signaling pathway, the condition leads to diffuse cystic lung destruction, recurrent spontaneous pneumothoraces, chylous pleural effusions, progressive dyspnea, and extrapulmonary...

It primarily affects women of reproductive age and occurs either sporadically or in association with tuberous sclerosis complex Pathophysiologically driven by hyperactivation of the mechanistic target of rapamycin signaling pathway, the condition leads to diffuse cystic lung destruction, recurrent spontaneous pneumothoraces, chylous pleural effusions, progressive dyspnea, and extrapulmonary manifestations such as renal angiomyolipomas and lymphangioleiomyomas