Lung function in patients with lymphangioleiomyomatosis

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., FEV 1 /VC − 56±20%, FEV1 − 64±31% pred., DL CO − 49±24% pred. DL CO , FEV 1 /VC, FEV 1 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 DL CO and FEV 1 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.