Elevated pulmonary arterial pressure in pulmonary lymphangiomyomatosis

Pulmonary lymphangiomyomatosis (LAM) is characterized by proliferation of smooth muscles around lymphatics and bronchovascular bundles, presenting clinical manifestations of obstructive or restrictive lung disease with greatly impaired gas exchange. To date, little attention has been paid in the literature to the pathophysiology of pulmonary hypertension related to this disorder. A case of a 39-year-old woman with the characteristic clinical and histological features of pulmonary LAM is presented. She was found to have moderate pulmonary hypertension. Possible mechanisms for the development of pulmonary hypertension are discussed.