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Pulmonary Lymphangioleiomyomatosis: Correlation of Ventilation-Perfusion Scintigraphy, Chest Radiography, and CT with Pulmonary Function Tests¹

¹ From the Departments of Diagnostic Radiology, Warren Grant Magnuson Clinical Center (N.A.A., E.C.J.) and Nuclear Medicine (C.C.C., R.D.N.), the Pulmonary-Critical Care Medicine Branch (S.C.C., J.M.), and the Office of Biostatistics Research, Division of Epidemiology and Clinical Applications (M.W.), National Heart, Lung, and Blood Institute, National Institutes of Health, Bldg 10, Rm 1C-660, 10 Center Dr, MSC 1182, Bethesda, MD 20892-1182. From the 1998 RSNA scientific assembly. Received December 9, 1998; revision requested December 30; final revision received May 20, 1999; accepted June 7. Address reprint requests to N.A.A. (e-mail: navila@nih.gov).

PURPOSE: To determine the findings on ventilation-perfusion (V-P) scintigrams, computed tomographic (CT) scans, and chest radiographs and correlate them with pulmonary function test results in patients with lymphangioleiomyomatosis.

MATERIALS AND METHODS: V-P scintigraphy, chest radiography, conventional and thin-section CT, and pulmonary function tests were performed in 39 patients. The images were graded on a scale of 0 (normal) to 3 (severely abnormal).

RESULTS: Imaging abnormalities were found on 92% of ventilation scintigrams, 92% of perfusion scintigrams, 79% of chest radiographs, 100% of CT scans, and 100% of thin-section CT scans. On ventilation scintigrams, 28 (72%) patients demonstrated a speckling pattern. On CT scans, all patients had pulmonary cysts. Univariate analysis showed that extent of disease on chest radiographs and CT scans, cyst size, V-P abnormalities, and degree of speckling were inversely correlated with forced expiratory volume in one second (FEV₁), diffusing capacity of lung for carbon monoxide, and the ratio of FEV₁ to forced vital capacity (FVC) (P < .01) but not with FVC and total lung capacity. Larger cyst size correlated with extent of disease at CT, but not significantly (P = .056).

CONCLUSION: Scintigraphic and radiologic abnormalities are seen in a majority of patients with lymphangioleiomyomatosis. On ventilation scintigrams, a frequently seen speckling pattern may be related to accumulation of radionuclide in pulmonary cysts—a hallmark of the disease at CT. Findings with each imaging modality correlate with certain pulmonary functions.

Index terms: Computed tomography (CT), comparative studies, 60.1211, 60.12118 • Computed tomography (CT), thin-section, 60.12118 • Lung, CT, 60.1211, 60.12118 • Lung, cysts, 60.312 • Lung, diseases, 60.3159, 94.829 • Lymphangiomyomatosis, 60.3159, 94.829 • Radiography, comparative studies, 60.11 • Radionuclide imaging, comparative studies, 60.12171, 60.12176

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