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Published ahead of print on September 4, 2003, doi:10.1164/rccm.200206-593OC

Am. J. Respir. Crit. Care Med., Volume 168, Number 12, December 2003, 1427-1431

A more recent version of this article appeared on December 15, 2003
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Submitted on June 25, 2002
Accepted on September 2, 2003

Maximal oxygen uptake and severity of disease in lymphangioleiomyomatosis

Angelo M Taveira-DaSilva1, Mario P Stylianou2, Carolyn J Hedin1, Arnold S Kristof1, Nilo A Avila3, Antoinette Rabel1, William D Travis4, and Joel Moss1*

1 Pulmonary-Critical Care Medicine Branch, National Heart, Lung and Blood Institute, Bethesda, MD, USA, 2 Office of Biostatistic Research, National Heart, Lung and Blood Institute, Bethesda, MD, USA, 3 Diagnostic Radiology, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, MD, USA, 4 Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC, USA

* To whom correspondence should be addressed. 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 forced expiratory volume in the first second (FEV1) are decreased, but, frequently, not in parallel with each other. Since 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, CT scans, lung function and histology. Maximal oxygen uptake (VO2 max) was decreased in 162 patients, of whom 28 did not reach anaerobic threshold (AT); 29 had low VO2 at AT and 54 developed hypoxemia. Hypoxemia occurred even in patients with near normal DLCO and FEV1. VO2 max decreased with increasing score of histological severity and was correlated with CT scans, use of oxygen, and resting PaO2. DLCO and FEV1, however, were the only significant predictors of VO2 max. We conclude that CPET uncovers the presence of exercise-induced hypoxemia and assists in grading disease severity and determining supplemental oxygen requirements in patients with LAM.
Key words: Interstitial lung diseases; Maximal oxygen uptake; Diffusion capacity; FEV1; CT scan; Lung histology; Oxygen therapy; Transplantation




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