Lymphangioleiomyomatosis

MORE than 50 years ago, von Stössel reported unique autopsy findings in a 43-year-old woman who had died of respiratory failure: her lungs showed diffuse cystic changes and lymphadenopathy on gross examination, and a striking proliferation of smooth muscle throughout the lungs microscopically. He labeled the process muscular cirrhosis of the lungs.¹ A few years later, Rosendal reported a similar case of “diffuse myomatosis and cyst formation in the lung.”² These were among the first reported cases of what is now known as pulmonary lymphangioleiomyomatosis (LAM). This rare disorder affects only women, usually causes serious respiratory impairment, and continues to. . .