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Lymphangioleiomyomatosis is a rare multisystem disease associated with genetic mutations. The disease usually occurs in women of reproductive age and is characterized by infiltration of immature smooth muscle cells into the lungs, airways, and axial lymphatic systems of the thorax and abdomen. The disease often destroys the lung parenchyma and produces air cysts. Cellular infiltration of lymphangioleiomyomatosis into the lymphatic axis can affect hilar lymph nodes, mediastinal nodes, and extrathoracic lymph nodes. The disease can cause lymphatic dilation in the lungs and thoracic ducts, causing chylous effusion in the pleural or abdominal cavities. Invasion of cells in the walls of pulmonary veins can lead to venous obstruction and pulmonary venous hypertension with hemoptysis. Most patients have cough, dyspnea, pneumothorax, hemoptysis, and abnormal lung function. Definitive diagnosis is usually based on histopathology and immunohistochemistry. We expect that, in the near future, quantitative blood tests for the growth factors VEGF-C and VEGF-D will become commonplace. Then the disease can be diagnosed more easily and in greater numbers.

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