Pulmonary lymphangioleiomyomatosis: Correlation of ventilation-perfusion scintigraphy, chest radiography, and CT with pulmonary function tests

PURPOSE: To determine the findings on ventilation-perfusion (V-P) scintigra ms, computed tomographic (CT) scans, and chest radiographs and correlate th em with pulmonary function test results in patients with lymphangioleiomyom atosis. MATERIALS AND METHODS: V-P scintigraphy, chest radiography, conventional an d thin-section CT, and pulmonary function tests were performed in 39 patien ts. 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%) pa tients demonstrated a speckling pattern. On CT scans, all patients had pulm onary cysts. Univariate analysis showed that extent of disease on chest rad iographs and CT scans, cyst size, V-P abnormalities, and degree of specklin g were inversely correlated with forced expiratory volume in one second (FE V1), 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 ca pacity. Larger cyst size correlated with extent of disease at CT, but not s ignificantly (P = .056). CONCLUSION: Scintigraphic and radiologic abnormalities are seen in a majori ty of patients with lymphangioleiomyomatosis. On ventilation scintigrams, a frequently seen speckling pattern may be related to accumulation of radion uclide in pulmonary cysts-a hallmark of the disease at CT. Findings with ea ch imaging modality correlate with certain pulmonary functions.