Сlinical - radiological features of lung’s lymphangioleiomyomatosis in long-term follow-up

Amosov V.I., Vasilieva M.A., Novikova L.N., Speranskaia A. A., Baranova O.P, Dzadzua D.V., Agafonov A.O.

Purpose. Lymphangioleiomyomatosis (LAM) is an orphan disease the long follow up of patients have not been studied. The use of various methods of radiological investigation (computed tomography (CT), high-resolution CT (HRCT), CT angiography, single-photon emission computed tomography (SPECT), positron emission computed tomography (PET-CT) and functional (SPECT, complex lung function examination) diagnostics allows to identify and evaluate the dynamics of the spectrum of thoracic and extra- thoracic changes in this disease. To assess the clinical and radiological investigation of thoracic and extra- thoracic changes in the dynamic observation of 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, radio-logical investigation and functional methods were used in the complex examination of pa-tients: X-ray in two projections, CT, HRCT, SPECT. If necessary, CT angiography was per-formed in 43 patients, PET-CT in 7 patients. Also patients underwent a complex lung function examination (CLFE) and echocardiography. Patients were observed in the dynamics (ob-servation period 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. Patients with a diffuse form were divided according CT features into 3 groups. I group - with single small cysts (14 patients), at primary SPECT in the patients of this group were no significant perfusion abnormalities, in the performance of CLFE in patients of this group, functional disturbances were absent or minimal, mainly due to deterioration of gas exchange conditions. For the I group, the presence of extra-thoracic manifestations of LAM was not noted. In 2 patients of first group there was a rapid increase of cysts number with the formation clinical and X-ray pattern identical for patients in third group. The second group consisted of 13 patients with multiple large cysts. In the SPECT for patients of the second group, presence of predominantly in the basal regions, aperfusion sites, corresponding with anatomical localization of cysts, CLFE revealed manifestations of severe bronchial obstruction in combination with moderate or significant diffusion disorders. Patients of this group were characterized by a slow, steady increase of changes. In the treatment of sirolimusum in 4 patients of this group, partial re-gression of lymphostasis in lung tissue was revealed, partial regression of chylothorax, chy-lous ascites, partial improvement of functional indices. The third group - with multiple small cysts (38 patients) had the most unfavorable course: during SPECT in patients of this group, pronounced perfusion disorders were noted: extended zones of hypoperfusion in both lungs, aperfusion sites in the subpleural sections from two sides. According to the results of CLFE, patients of this group had mixed disorders of respiratory mechanics with a sharp deteriora-tion in the conditions of gas exchange in the lungs. The follow up in 31% of cases (12 pa-tients) showed progression of the process, frequent and severe exacerbations. During the treatment with sirolimusum, 7 patients of this group showed partial regression of lymphos-tasis in lung tissue, partial regression of chylothorax, chylous pericarditis, chylous ascites, partial improvement of functional parameters. 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, uterus fibroma and leiomyoma in 27 patients, liver in 3 patients, and extraorganic leiomyomas in 16 patients. Comorbid processes were defined in 25 patients: alveolar-interstitial pulmonary edema (as a result of heart failure or RDS in 4 patients), pulmonary embolism (in 11 patients), inflammation (in 10 patients). Conclusions. The accumulation of experience in clinical - radiological features of LAM patients allows to evaluate the dynamics of its thoracical and extra-thoracical manifes-tations, comorbid processes, to identify unfavorable forms of LAM flow, to identify complica-tions and comorbid processes that are important for the management of these patients.
I.P. Pavlov First St.-Petersburg State Medical University. Saint Petersburg, Russia.







Keywords:computed tomography, lymphangioleiomyomatosis (LAM), treatment.


Corresponding author:Vasilieva M.A. e-mail: Этот e-mail адрес защищен от спам-ботов, для его просмотра у Вас должен быть включен Javascript


For citation: Amosov V.I., Vasilieva M.A., Novikova L.N., Speranskaia A. A., Baranova O.P, Dzadzua D.V., Agafonov A.O. Сlinical - radiological features of lung’s lymphangioleiomyomatosis in long-term follow-up. REJR 2018; 8(1):102-118. DOI:10.21569/2222-7415-2018-8-1-102-118.

Received: 01.02.2018 Accepted: 02.03.2018