@article{95ede16e3cde4efb9d1c9e236c8f6edf,
title = "Pulmonary lymphangioleiomyomatosis: Quantitative analysis of lesions producing airflow limitation",
abstract = "The lungs of three patients dying of lymphangioleiomyomatosis (LAM), which in two of the patients was associated with tuberous sclerosis, were studied to characterize better the sites of airflow limitation in this condition. Quantitative studies showed that small airways were narrowed and collapsed because of the surrounding emphysema, but few airways contained excess smooth muscle. These findings suggest that the airspace lesions are more important than muscular proliferation in small airways in producing airflow limitation. In the two patients who had LAM with tuberous sclerosis, sex steroid assays were negative. Pleurodesis controlled pleural effusions in all three patients but may have contributed to reductions in lung volume.",
author = "Sobonya, {Richard E.} and Quan, {Stuart F.} and Fleishman, {Jay S.}",
note = "Funding Information: Lymphangioleiomyomatosis (LAM) is an uncommon pathologic entity that particularly affects the hmgs, with variable involvement of lymph nodes, lymphatics, and the thoracic duct. Puhnonary LAM is characterized histologically by diffuse proliferation of smooth muscle, with variable proliferation of small blood vessels and lymphatics. Pttlmonary LAM typically is seen either in women of child-bearing age with no other disease I-5 or in patients with tuberous sclerosis. 5-8 Most authors believe that the pathologic features of puhnonary LAM in patients with tuberous sclerosis do not differ from those in patients without tuberous sclerosis, 3,9 ahhough others have noticed subtle differences. 5 When part of the spectrum of the pathologic changes of tttberous sclerosis, LAM may affect men or women, although women are more commonly affected; LAM develops in approximately 1 per cent of persons with tuberous sclerosis. 8 Whether LAM occurring by itself is a forme fruste of the tuberous sclerosis complex has not been deter-minedP-lt The typical clinical course in LAM is that of progressive dyspnea, often punctuated by episodes of chylothorax or pneumothorax, with eventual death in respiratory failure. Chest radiographs in well-established disease typically show cyst-like spaces, usually referred to as honeycombing, and lung volumes appear to be increased. Despite the radiographic ap- Medicine and the Division of Respiratory Sciences, University of Arizona College of Medicine, and the ~Department of Pathology, Kino CommunityH ospital,T ucson, Arizona. Revisiona ccepted for publication May 6, 1985. St, pported by Specialized Center of Research grant IlL 14136 from the National tteart, Lvng and Blood Institute, Bethesda, Maryland.",
year = "1985",
month = nov,
doi = "10.1016/S0046-8177(85)80180-5",
language = "English (US)",
volume = "16",
pages = "1122--1128",
journal = "Human Pathology",
issn = "0046-8177",
publisher = "W.B. Saunders Ltd",
number = "11",
}