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


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 (FEV(1)), diffusing capacity of lung for carbon monoxide, and the ratio of FEV(1) 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).


CONCLUSIONS
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.
Avila
N A
NA
Department of Diagnostic Radiology, Warren Grant Magnuson Clinical Center, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892-1182, USA. navila@nih.gov
Chen
C C
CC
Chu
S C
SC
Wu
M
M
Jones
E C
EC
Neumann
R D
RD
Moss
J
J
D000328:Adult; D000704:Analysis of Variance; D002248:Carbon Monoxide; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000000981:diagnostic imaging; D008875:Middle Aged; D011653:Pulmonary Diffusing Capacity / Q000502:physiology; D013902:Radiography, Thoracic; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals; D012129:Respiratory Function Tests; D016284:Technetium Tc 99m Pentetate; D014057:Tomography, X-Ray Computed; D014109:Total Lung Capacity / Q000502:physiology; D014692:Ventilation-Perfusion Ratio; D014797:Vital Capacity / Q000502:physiology
D019275:Radiopharmaceuticals; D002248:Carbon Monoxide; D016284:Technetium Tc 99m Pentetate