Lymphangioleiomyomatosis

Abstract

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 frustrate clinicians attempting to treat it.