Am. J. Respir. Crit. Care Med., Volume 164, Number 6, September 2001, 1072-1076

Reversible Airflow Obstruction, Proliferation of Abnormal Smooth Muscle Cells, and Impairment of Gas Exchange as Predictors of Outcome in Lymphangioleiomyomatosis

ANGELO M. TAVEIRA-DaSILVA, CAROLYN HEDIN, MARIO P. STYLIANOU, WILLIAM D. TRAVIS, KASUHIRO MATSUI, VICTOR J. FERRANS, and JOEL MOSS

Pulmonary-Critical Care Medicine Branch, Office of Biostatistics Research, and Pathology Section, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland; and Pulmonary and Mediastinal Department, Armed Forces Institute of Pathology, Washington, DC

Lymphangioleiomyomatosis (LAM) is a rare disease, occurring in women, characterized by cystic degeneration of the lungs, abdominal tumors, and proliferation of abnormal smooth muscle cells. Lung function abnormalities consist of impairment of the diffusion capacity (DL_CO) and airflow obstruction. The objective of this study was to correlate the functional impairment with histologic measures of disease severity to identify predictors of disease outcome. Lung function of 143 patients and lung biopsies of 74 of these patients were reviewed for evidence of airway disease and scoring of disease severity. A positive response to bronchodilators was associated with more severe airflow obstruction, a predominantly solid pattern of LAM lesions in the lung biopsy, and greater rate of decline in expiratory flow. Airway inflammation, present in 61% of the lung specimens, was not associated with reversible airway obstruction and did not correlate with the severity of airflow obstruction. DL_CO correlated best with the LAM histologic score (LHS), a demonstrated predictor of outcome. We conclude that reversible airway obstruction is found in LAM patients with accelerated loss of lung function and a predominantly solid pattern of LAM lesions. Impairment of DL_CO correlates with LHS, a predictor of survival and time to lung transplantation.

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