Lung function in patients with lymphangioleiomyomatosis
https://doi.org/10.36604/1998-5029-2021-79-21-31
Abstract
Aim. To assess the functional parameters of the respiratory system and their impact on the survival of patients with lymphangioleiomyomatosis (LAM).
Materials and methods. A study of lung function (spirometry, bodyplethysmography, diffusion test) was carried out in 131 patients with LAM (mean age 41±10 years), who were under observation and treatment in medical institutions in Moscow (Pulmonology Scientific Research Institute, Sechenov University, Moscow City Clinical Hospital named after D.D.Pletnev). The diagnosis of the disease was made based on the diagnostic criteria for LAM, formulated by a group of experts at the initiative of the European Respiratory Society in the guidelines for the diagnosis and treatment of LAM.
Results. The mean VC value was 95±25% pred., FEV1 /VC − 56±20%, FEV1 − 64±31% pred., DLCO − 49±24% pred. DLCO, FEV1 /VC, FEV1 and VC were reduced in 87, 76, 70 and 27% of patients, respectively. The obstructive ventilatory defect was detected in the overwhelming majority of cases − in 75%, restrictive ventilatory defect − in 4 patients, mixed defect − in 1 patient. In 28 (21%) patients, there were no pulmonary ventilation disorders. The Cox regression model showed that baseline DLCO and FEV1 are the main factors determining the risk of death.
Conclusion. In patients with LAM, the obstructive ventilatory defect predominates, the restrictive ventilatory defect is quite rare. The lung diffusion capacity is reduced in 87% of patients with LAM. Parameters of lung function are closely related to survival.
Supporting agencies: This study was not sponsored
About the Authors
A. V. Chernyak
Pulmonology Scientific Research Institute of Federal Medical and Biological Agency;
Moscow City Clinical Hospital named after D.D.Pletnev,
Russian Federation
Russian Federation
Alexander V. Chernyak, MD, PhD (Med.), Head of the Laboratory of Functional and Ultrasonic Research Methods; Doctor of Functional Diagnostics of the Department of Functional and Ultrasound Diagnostics
28 Orekhovuy Boulevard, Moscow, 115682;
32 11-Parkovaya Str., Moscow, 105077
M. A. Makarova
Pirogov Russian National Research Medical University (Pirogov Medical University);
Moscow City Clinical Hospital named after D.D.Pletnev
Russian Federation
Russian Federation
Marina A. Makarova, MD, PhD (Med.), Associate Professor of the Department of Hospital Therapy of the Pediatric Faculty; Pulmonologist of the Department of Respiratory Medicine
1 Ostrovityanova Str., Moscow, 117997;
2 11-Parkovaya Str., Moscow, 105077
S. N. Avdeev
I.M.Sechenov First Moscow State Medical University (Sechenov University);
Pulmonology Scientific Research Institute of Federal Medical and Biological Agency
Russian Federation
Russian Federation
Sergey N. Avdeev, MD, PhD, DSc (Med.), Professor, Corresponding member of RAS, Head of the Pulmonology Department of the Medical Faculty; Head of the Clinical Department
8 bld. 2 Trubetskaya Str., Moscow, 119991;
28 Orekhovuy Boulevard, Moscow, 115682
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For citations:
Chernyak A.V., Makarova M.A., Avdeev S.N. Lung function in patients with lymphangioleiomyomatosis. Bulletin Physiology and Pathology of Respiration. 2021;(79):21-31. (In Russ.) https://doi.org/10.36604/1998-5029-2021-79-21-31
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