Lymphangioleiomyomatosis is a slowly progressive lung disease that typically results in cystic lung destruction and predominantly affects women, especially of reproductive age. Cystic destruction of the lung with progressive pulmonary dysfunction and the presence of abdominal tumors are the main characteristics of this disease. This article outlines the diagnosis and treatment options of Lymphangioleiomyomatosis. Lymphangioleiomyomatosis (LAM) is a rare disease characterized by cystic lung destruction, lymphatic involvement, and renal angiomyolipomas.
It affects 5 to 9 million women, worldwide. Although men and children have been reported with LAM, this is highly unusual and LAM is one of the most sex-specific diseases described suggesting the involvement of female hormones in disease pathogenesis. The natural history of LAM is highly variable but most women with LAM develop a risk of…
