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Published ahead of print on September 4, 2003, doi:10.1164/rccm.200206-593OC
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American Journal of Respiratory and Critical Care Medicine Vol 168. pp. 1427-1431, (2003)
© 2003 American Thoracic Society

Maximal Oxygen Uptake and Severity of Disease in Lymphangioleiomyomatosis

Angelo M. Taveira-DaSilva, Mario P. Stylianou, Carolyn J. Hedin, Arnold S. Kristof, Nilo A. Avila, Antoinette Rabel, William D. Travis and Joel Moss

Pulmonary-Critical Care Medicine Branch and Office of Biostatistics Research, National Heart, Lung, and Blood Institute; Department of Diagnostic Radiology, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland; and Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC

Correspondence and requests for reprints should be addressed to Joel Moss, M.D., Ph.D., Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Building 10, Room 6D05, MSC 1590, Bethesda, MD 20892–1590. E-mail: mossj{at}nhlbi.nih.gov

Lymphangioleiomyomatosis (LAM), a disease that occurs primarily in women, is characterized by cystic lung lesions causing respiratory failure, which may require lung transplantation. Lung diffusion (DLCO) and/or FEV1 are decreased, but frequently not in parallel with each other. Because cardiopulmonary exercise testing (CPET) provides information that is not obtainable from resting cardiopulmonary tests, we performed CPET in 217 LAM patients and correlated exercise data with clinical markers of severity, computed tomography scans, lung function, and histology. O2max was decreased in 162 patients, of whom 28 did not reach anaerobic threshold; 29 had low oxygen uptake at anaerobic threshold, and 54 developed hypoxemia. Hypoxemia occurred even in patients with near normal DLCO and FEV1. O2max decreased with an increasing score of histologic LAM severity and was correlated with computed tomography scans, the use of oxygen, and resting PaO2. DLCO and FEV1, however, were the only significant predictors of O2max. We conclude that CPET uncovers the presence of exercise-induced hypoxemia and assists in grading the severity of disease and determining supplemental oxygen requirements in patients with LAM.

Key Words: interstitial lung disease • maximal oxygen uptake • diffusion capacity • computed tomography scan • lung histology




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