La linfangioleiomiomatosis pulmonar: Dos caras de una compleja enfermedad

Pulmonary Lymphangioleiomyomatosis:two sides of a complex diseaseLymphangioleiomyomatosis (LAM) is a rare systemic disease associated with interstitial lung involvement.It is considered a neoplastic disease characterized by abnormal proliferation of smooth muscle cells in the lung, forming cysts and causing severe respiratory disorders.Its diagnosis is based on specific computed tomography findings and high serum levels of vascular endothelial growth factor type D (VEGF-D).Sirolimus, an mTOR inhibitor, is the first-line therapy and has been shown to stabilize lung function and reduce symptoms.We present two cases of young women with LAM who differ in the severity of their clinical presentation.The first case presents a patient with a complicated clinical course with multiple relapses and the need for sirolimus therapy.The second case presents a patient with a less aggressive form, without the need for treatment with an mTOR inhibitor.