lymphangioleiomyomatosis(redirected from Pulmonary lymphangioleiomyomatosis)
a progressive disorder of women of child-bearing age, marked by nodular and diffuse interstitial proliferation of smooth muscle in the lungs, lymph nodes, and thoracic duct. Called also lymphangioleiomyomatosis.
A rare disorder of unknown etiology seen in women of reproductive age and in patients of either sex with tuberous sclerosis. Pulmonary complications are due to hamartomatous proliferation of smooth muscle cells preferentially along bronchovascular structures resulting in obliteration of the airways and consecutive development of cysts in the lungs. Usually progressive, leading to death from respiratory failure. Treatment by lung transplantation has been successful.