Аннтотация

© 2018 Russian Electronic Journal of Radiology. All rights reserved. Lymphangioleiomyomatosis (LAM) is an orphan disease, which patterns have not been fully studied. The use various methods of diagnosing (computed tomography, HRCT, CT angiography, SPECT, PET-CT) allows to identify and to evaluate the spec-trum of thoracic and extra- thoracic changes in this disease. Purpose. To evaluate the clinical and radiation characteristics of thoracic and extra- thoracic changes in patients with LAM. Materials and methods. The study included 83 patients with morphologically con-firmed LAM. The average age of patients was 42.2 ± 6.3 years (w / m - 83/0). Clinical and X-ray were used in the complex examination of patients: radiography in two projections, com-puted tomography (CT), high-resolution CT (HRCT), and single-photon emission computed tomography (SPECT). If necessary: CT angiography - in 43 patients, PET-CT in 7 patients. Also, patients underwent a complex lung function examination (CLFE) and echocardiog-raphy. All patients were observed in dynamics (observation was 6.1 ± 4.2 years). Results. Analysis of the results of clinical and radiological investigation revealed the following variants of pulmonary manifestations of LAM: the nodular form was verified in 14 patients, diffuse in 65 patients and mixed in 4 patients. The diffuse form was divided ac-cording to the CT scan into 3 groups. The first group - with sporadic small cysts (14 pa-tients). During SPECT in patients of this group, no significant perfusion disorders were de-tected, while performing CLFE in patients of this group revealed manifestations of bronchial obstruction. The second group - with multiple large cysts (13 patients), during SPECT for the patients of this group was characterized by the presence, mainly in the central parts of lungs focuses of aperfusion, corresponding with anatomical localization of cysts. CLFE re-vealed pronounced manifestations of bronchial obstruction in combination with diffusion disorders. The third group - with multiple small cysts (38 patients) was characterized by the most unfavorable course: during SPECT in patients of this group, pronounced perfusion disorders were noted: extended hypoperfusion zones in both lungs, aperfusion areas in sub-pleural sections from both sides, according to the results of CLFE in this group of patients obstructive violations of breathing mechanics with a sharp deterioration of gas exchange conditions in the lungs (decrease in FEV1, diffusive capacity of the lungs) were revealed. Complications of the disease were determined in the majority of patients (54 patients - 63%): pneumothorax in 52 patients, chylothorax in 16 patients, pulmonary hypertension in 18 patients, pulmonary hemorrhage in 2 patients, lymphostasis in 9 patients. Extrathoracic changes were detected in 52 patients: kidney leiomyoma in 14 patients, uterine fibroids and uterine fibroids in 27 patients, liver in 3 patients, and extraorganic leiomyomas in 16 pa-tients. Conclusions. The use of a comprehensive clinical and radiation survey allows us to identify the signs of LAM in the early stages of the disease, in some cases avoid the use of surgical biopsy associated with a high risk of complications. The selection of various vari- ants of the flow of the diffuse form of LAM on the basis of the CT features of the picture, which correlate with functional disorders, allows the development of differentiated ap-proaches to therapeutic tactics and determining the prognosis of the disease.