Body plethysmography (synonyms: plethysmography, whole body plethysmography) is an important method for evaluation of the ventilation function of the respiratory system. This method can be used to measure static lung volumes and airway resistance. Methods. This document on body plethysmography was developed by a group of experts from the Russian Respiratory Society and the Russian Association of Specialists in Functional Diagnostics. All experts have experience in conducting research in accordance with quality criteria, analyzing the test results, and developing national standards. Results. Technical standards for body plethysmography were recommended, including criteria for the measurement quality and assessment of the test quality. The experience and knowledge of the expert group members were used in developing these recommendations. Conclusion. The article presents standards for measuring static lung volumes and airway resistance together with an algorithm for interpreting the results. This should contribute to improving the quality of measurements and uniformity in data interpretation.

Despite active discussion in different countries of the world about possible reduction of the amount of basic therapy of cystic fibrosis due to the use of CFTR-modulators, in particular mucolytics, many patients continue mucolytic inhalation therapy, including mannitol. The aim. A retrospective evaluation of the tolerability of the inhaled mannitol (Bronchitol Pharmaxis, GEN Ilac, Turkey) in children with cystic fibrosis (cwCF). Methods. 142 cwCF aged from 6 to 17 years (62 boys) who underwent inpatient treatment at the Separate Structural Unit Russian Children’s Clinical Hospital of the Federal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian Federation from 2021 to 2023 were administered the initial dose assessment BIDA test. Results. Most cases demonstrated good tolerability and pronounced mucolytic effect when using the study drug. Isolated cases of intolerance (6.3%) in the form of acute bronchobstructive syndrome were observed. Conclusion. The presented evaluation of the effect of an initial dose of inhaled mannitol in a sufficiently large cohort of cwCF of different ages clearly illustrates the high safety and efficacy profile of the study drug.

Reference values are needed to interpret spirometry results. The ECCS 1993 reference equations are widely used in Russia. A small proportion of medical facilities have implemented the GLI (2012) reference equations. In 2022, ERS/ATS published recommendations for interpreting spirometry results, which recommend assessing the presence of obstructive disorders by a decrease in FEV1/FVC below the 5th percentile (LLN) and the severity of lung function impairment using a z-score.

The aim. To compare spirometry results in adult patients with cystic fibrosis (CF) in Russia using ECCS and GLI reference values.

Methods. Spirometry in 161 CF adults (70 male/91 female, mean age – 26.3 ± 7.6 years) was used for the comparison. The predicted values, % of predicted values (%pred.) and z-score were calculated. The t-tests were used to compare the ECCS and GLI equations.

Results. Obstructive disorders were detected in 127 (79%) CF patients according to ERS/ATS criteria. Predicted GLI and LLN values were higher for FVC and FEV1, but lower for FEF25–75 and FEF75 in both genders. The %pred and z-score for GLI were significantly lower for FVC and FEV1 compared with the %pred and z-score for ECCS: 79.3%pred and –1.75 vs 83.6%pred and –1.31 (p < 0.001), and 58.7%pred and –3.30 vs 61.2%pred and –3.11 (p < 0.001), respectively. A decrease in FVC and FEV1 was detected using the GLI and ECCS equations in 78 (48.4%) and 130 (80.7%) patients and vs 62 (38.5%) and 124 (77.0%) patients, respectively. In addition, the number of patients with severe impairments in both FVC increased when assessed by GLI versus ECCS (mild: 15% vs 11%, moderate: 22% vs 20%, severe: 12% vs 7%, respectively) and FEV1 (mild: 15% vs 14%, moderate: 23% vs 21%, severe: 43% vs 42%, respectively).

Conclusion. In adult patients with cystic fibrosis, the use of GLI equations is associated with increased detection of both lung function impairment and more severe changes in the spirometric parameters FVC and FEV1. The frequency of lowered FVC and FEV1 statistically significantly increases with the use of GLI. Forced spirometry and detection of a decrease in FEV1/FVC are sufficient to diagnose obstructive disorders.

Primary ciliary dyskinesia (PCD) is a rare hereditary disease belonging to the group of ciliopathies. PCD is caused by a defect in the ultrastructure of the cilia of the respiratory epithelium and similar structures (sperm flagella, fallopian tube villi, ventricular ependyma) with a prevalence of 1:10,000 to 1:30,000 people. One of the main problems associated with this condition is frequent recurrent bacterial infections, which lead to decreased lung function and the development of bronchiectasis.

The aim was to study the microbial landscape of the respiratory tract of patients with PCD.

Methods. We studied the medical history and bacteriological assays of 170 throat, nasal, and sputum samples from 64 patients diagnosed with PCD. virological studies on pathogens of acute respiratory viral infection, using polymerase chain reaction with reverse transcription, and MALDITOF mass spectrometry were used to identify microorganisms.

Results. The assay identified 46 bacterial species, 4 viral species, and representatives of 2 fungal genera. The predominant bacteria were Staphylococcus aureus (62.5%) and Pseudomonas aeruginosa (26.6%). The identified fungi were Candida spp. and Aspergillus spp. Viruses (Adenoviridae, Coronaviridae, Rhinoviridae) were detected in 7 patients in association with representatives of normal microflora and clinically significant microorganisms such as S. aureus, Klebsiella pneumoniae, P. aeruginosa. Achromobacter spp., Stenotrophomonas maltophilia, Acinetobacter spp., and Moraxella spp. were isolated in individual cases in patients of all age groups.

Conclusion. The respiratory microflora was characterized by a species diversity of pathogens and was mixed in 79.7% of patients. A viral-bacterial associations was observed in 10.9% of patients. S. aureus was the predominant pathogen in the group of children and adolescents (58.3% and 82.6%, respectively), whereas P. aeruginosa predominated (52.9%) in the adult patients.

The interest in medical gases for the correction of respiratory failure (RF) has been growing over recent years. However, there are virtually no scientific studies of combination therapy for exacerbation of chronic obstructive pulmonary disease (COPD), which is a combination of non-invasive ventilation (NIV) with thermal heliox (t-He/O₂), nitric oxide (NO₂) and molecular hydrogen (H₂), in the Russian Federation and in the world.

The aim of the study was to investigate the efficacy and safety of the combined use of t-He/O₂, NO and H₂ in patients with exacerbation of COPD complicated by hypoxemic, hypercapnic RF and secondary pulmonary hypertension (PH) in the post-COVID period.

Methods. The randomized, comparative, controlled, parallel-group, prospective study included 100 patients (52 men, 48 women) with exacerbation of COPD categories C and D according to the criteria of the Global Initiative for Chronic Obstructive Lung Disease (GOLD, 2021 – 2023), with hypoxemic, hypercapnic respiratory failure and PH, who had COVID-19 (COronaVIrus Disease 2019) complicated by pneumonia caused by SARS-CoV-2 (Severe Acute Respiratory Syndrome-related CoronaVirus 2) before the hospitalization. Patients with similar demographic, clinical, and functional parameters, who received, along with standard drug therapy, NIV in the spontaneous-forced biphasic positive airway pressure (BiPAP S/T) mode and oxygen (O₂) to maintain blood hemoglobin oxygen saturation (SpO₂) in the range of 95–97% according to GOLD (2021 – 2023), were divided into 5 groups: patients of the 1^st (main) group (n = 22: 12 men, 10 women) received t-He/O₂, NO, and H₂ sequentially; the 2^nd group (n = 20: 10 men, 10 women) – t-He/O₂ and NO; the 3^rd group (n = 20: 11 men, 9 women) – t-He/O₂ and H₂; the 4^th group (n = 18: 10 men, 8 women) – NO and H₂; the 5^th group (control) (n = 20: 9 men, 11 women) – only standard drug therapy, NIV and O₂. Changes in the clinical condition of the patients, gas exchange in the lungs, acid-base balance, left-to-right shunt fraction, hemodynamic parameters, and exercise tolerance (ET) were assessed.

Results. Not only clinical condition of the patients, but also the arterial blood gas composition, acid-base balance and intrapulmonary blood shunt fraction, hemodynamics and ET were improved in the main group compared to those in patients who received inhalation therapy with medical gases separately and the control group.

Conclusion. The combination of t-He/O₂, NO and H₂ combined with pathogenetic therapy and NIV in patients with exacerbation of COPD complicated by hypoxemic, hypercapnic DN and secondary PH in the post-COVID period is safe and more effective compared to administering each medical gas separately. The clinical condition of patients improves, signs of hypoxemia and hypercapnia, vascular endothelial dysfunction, and metabolic disorders are reduced with the combined therapy. An increase in ET was also noted due to the normalization of gas exchange in the lungs, increased oxygen delivery to tissues, a decrease in the shunt fraction, and restoration of metabolism.

Chronic obstructive pulmonary disease (COPD) is one of the most widespread diseases with a significant socio-economic impact. Nevertheless, properly selected therapy makes it possible to effectively control the course of the disease. Fixed triple combinations are most effective in the severe forms. The list of drugs in this group is constantly expanding, which necessitates selecting the therapeutic approach with an optimal ratio of clinical effectiveness and economic effect of therapy.

The aim of the study was to conduct a clinical and economic analysis of the use of a fixed combination of budesonide + glycopyrronium bromide + formoterol (BGF) in patients with COPD receiving triple combinations of long-acting anticholinergic drugs/long-acting β2-agonists/inhaled corticosteroids (LAMA/LABA/ICS).

Methods. The triple fixed combination of vilanterol + umeclidinium bromide + fluticasone furoate (VUF) was selected as the active comparison. According to the adjusted indirect comparison data, the relative risk of death associated with BGF was 0.61 (95% CI 0.38 – 0.95) compared to VUF. A mathematical model of the COPD course against the use of the compared alternatives was proposed based on the adjusted indirect comparison data. The model was used to calculate the average number of life years and direct medical costs of the main drug therapy when using the considered alternatives over a modeling horizon of 3 years.

Results. The results of modeling the effectiveness of the considered alternatives conclude that therapy with BGF within the 3-year study horizon provides an additional 0.044 years of life compared with the use of VUF. Taking into account the termination of therapy due to death from all causes, the savings will amount to 47,091 rubles when using BGF in a 3-year horizon compared to the use of VUF (108,111 rubles vs 155,202 rubles). Thus, therapy with BGF is more effective and less expensive (dominant therapy).

Conclusion. From the perspective of the state payer’s budget, the BGF fixed combination is preferred over VUF for patients with COPD who are candidates for triple therapy and can be recommended for use in the Russian healthcare system.

Bronchial asthma is one of the most common chronic non-infectious inflammatory diseases of the respiratory tract. Asthma is characterized by significant phenotypic and endotypic heterogeneity, which complicates the selection of therapy in patients.

The aim was to assess the clinical and economic feasibility of tezepelumab compared to the standard therapy in patients with severe asthma (SA) in the Russian Federation.

Methods. A clinical and economic analysis of the use of tezepelumab in combination with the standard therapy was conducted in comparison with the standard therapy for SA only. The study period was 5 years. The study was performed using cost-effectiveness analysis. The costs were calculated with a mathematical model adapted to the health care conditions in the Russian Federation. The study considered both direct costs (for drug therapy, hospitalization, outpatient care) and indirect costs (disability payments and losses of gross domestic product – GDP).

Results. Over a 5-year modeling horizon, the use of tezepelumab added 0.413 years of life and 0.352 years of quality life compared to the standard therapy, while reducing total costs by RUB 2,069,322 due to a reduction in GDP losses due to mortality and temporary disability. As a result of using tezepelumab, the number of days of disability was reduced almost 2-fold compared to the standard therapy (115 days vs 177 days). Costs in the 1st year of modeling were calculated separately, showing a decrease in total costs by RUB 245,393 when using tezepelumab. Thus, therapy of patients with SA with tezepelumab is clinically justified because it leads to an increase in the working life expectancy of patients with asthma. It is also economically feasible, since it leads to a reduction in indirect costs and GDP losses for the state.

Conclusion. The use of tezepelumab in the patients with SA is pharmacoeconomically effective compared to the standard therapy in the Russian Federation.

Budesonide/albuterol (salbutamol) combination (BDA) is a fixed combination of salbutamol (albuterol) and budesonide in the form of a metereddose aerosol for inhalation, the efficacy and safety of which have been demonstrated by several international phase III clinical trials in adults, adolescents, and children with asthma. This clinical trial is part of a program to study BDA in asthma patients with exercise-induced bronchospasm (EIB).

The aim of the study was to evaluate the efficacy and safety of budesonide/salbutamol compared with placebo in patients with asthma and EIB.

Methods. Adult patients with EIB (defined as a fall in forced expiratory volume in 1 second (FEV1) ≥ 20% from pre-exercise (PE) values) participating in a double-blind, crossover study were randomized to a single dose of study medication in the sequence BDA (160/180 mcg)/placebo (n = 31) or the reverse sequence (n = 33). Serial FEV1 measurements were made 5 min before and 30 min after drug administration (i.e., 5 min before the treadmill exercise challenge (ECT); baseline) and 5, 10, 15, 30, and 60 min after PE. The primary efficacy endpoint was the maximum percentage fall in FEV1 from baseline at 60 min after ECT.

Results. With BDA, the mean maximum percentage of FEV1 decline within 60 minutes after ECT was 6.95% vs 22.4% with placebo (the difference between the drugs was 17.52% (95% confidence interval was 15.25 – 19.78%; p < 0.001). The protective effect in preventing EIB (determined by the maximum decline in FEV1 after ECT < 10%) was achieved in a greater number of patients after BDA compared with placebo (75.0% and 3.1%, respectively; p < 0.001). The effect was demonstrated both in the overall study population and in the subpopulations of patients who received and did not receive inhaled corticosteroids. The single dose of BDA showed satisfactory tolerability in the study.

Conclusion. The study results confirmed the possibility of using BDA (160/180 mcg) as a new and effective pharmacological method to prevent EIB.

Lymphangioleiomyomatosis (LAM) is a rare disease with a predominant diffuse cystic lesion of the lungs in women. Currently, with the widespread availability of computed tomography (CT), the number of patients with LAM is increasing significantly. All this highlights the importance and significance of knowledge about this orphan disease. In clinical practice, LAM occurs either as an independent disease, sporadic LAM, or as LAM associated with tuberous sclerosis complex (TSC – LAM), a major sign of tuberous sclerosis. The aim was to present an overview of the main common and different manifestations of both variants of LAM.

Conclusion. Despite the common clinical, radiological and morphological features, both LAM variants have significant differences. The article discusses in detail the main features of different forms of LAMs.

The COVID-19 pandemic has brought to the forefront the use of telemedicine in healthcare delivery during a public health emergency for remote patient assessment and care for patients already infected with coronavirus or those afraid of contracting COVID-19 through face-to-face contact. The need for telemedicine has increased during this period, including among patients with chronic obstructive pulmonary disease (COPD), who are at increased risk of infection and hospitalization.

The aim of this review was to study the feasibility and effectiveness of using various telemedicine methods in managing patients with COPD in terms of disease control and the impact of these interventions on changing patients’ behavior in terms of self-management of this pathology.

Results. The COVID-19 pandemic has dramatically accelerated the use and rapid development of digital technologies, including in relation to patients with COPD. Short-term improvements in quality of life were noted in these patients with COPD, but it is unclear whether these are maintained in the long term, across multiple interventions, and for specific disease severity subgroups. However, digital interventions have little effect on COPD exacerbation rates. Depending on patient vulnerability and COPD severity, some patients may require individual care rather than group interventions.

Conclusion. Looking ahead, the challenge is to ensure that telemedicine remains relevant and that telemedicine visits are maintained beyond the COVID-19 pandemic. This poses specific communication challenges that healthcare professionals should be prepared to address. Additional research in this area will allow for more robust conclusions on the use of digital interventions and understanding the attitudes of different patient groups towards these new approaches to COPD care.

The success of antibacterial therapy has overshadowed non-drug pathogenetic methods of tuberculosis treatment. However, the growth of drug resistance requires not only the search for new reserve drugs for the treatment of the disease but also the active study and conscious use of longknown non-drug methods.

The aim of the work was to familiarize doctors with the main directions of non-drug pathogenic treatment and to create motivation for a more detailed study of individual directions and their implementation in clinical practice.

Conclusion. The use of most non-drug treatment methods is justified from the point of view of modern understanding of the pathogenesis of the disease. Evidence criteria have to be developed for a more complete study of their effectiveness and subsequent inclusion in clinical guidelines.

Intrathoracic lymph node tuberculosis (ITLNTB), or tuberculous bronchoadenitis, is increasingly diagnosed in middle aged patients in the era of spreading human immunodeficiency virus (HIV) infection. The difficulty of early diagnosis lies in the absence of specific symptoms at the initial stages of the disease. Often, the diagnosis can be verified only after complications develop.

The aim of the study was to analyze a clinical case of diagnosing ITLNTB in the practice of a pulmonologist.

Methods. The article presents the results of general clinical, laboratory, and instrumental research methods, and other materials of a patient with a long previous history of respiratory symptoms.

Results. According to the results of the diagnostic minimum at the initial examination, no data for tuberculosis were found, and the antibacterial therapy for 7 days had no effect. Intoxication syndrome persisted. Computed tomography (CT) of the chest organs detected fistulas from the paratracheal lymph nodes to the esophagus and bronchi. Mycobacterium tuberculosis was detected in the bronchoalveolar lavage fluid using the Ziehl – Neelson staining method.

Conclusion. Despite the modern extensive diagnostic capabilities, such as CT, fibrobronchoscopy (FBS), and fibrogastroduodenoscopy, the diagnosis of tuberculosis is still highly challenging due to the numerous “masks”, long-term latent development of symptoms, and clinical manifestations only with the development of a fistula. FBS is recognized as the primary method for diagnosing tuberculosis of the trachea and bronchi. It not only visualizes the mucosal lesion, but also allows obtaining material for microbiological and histological examination.

Interstitial lung diseases (ILD) are one of the most frequent drug-induced disorders of the respiratory system. Drug-induced interstitial lung diseases (DIILD) account for 3% to 6% in the structure of all ILD. The diagnosis of DIILD requires the exclusion of other possible causes of ILD, especially in oncological and immunocompromised patients. The diagnostic challenges are associated with rare cases of drug-induced pneumopathy or with the use of several potentially relevant drugs, as well as in combination with radiotherapy in cancer patients.

The aim of the report was to demonstrate a rare clinical case requiring differential diagnosis between radiation pneumonitis and DIILD in a 53-year-old woman undergoing treatment for breast cancer. This case illustrates the difficulties in the differential diagnosis of tamoxifen-induced interstitial lung lesions. The diagnosis of tamoxifen-induced organizing pneumonia (OP) was made on the basis of exclusion of other causes, primarily infectious lesions, recurrent nature of symptoms, and X-ray picture of bilateral OP on repeated administration of tamoxifen.

Conclusion. An awareness and suspicion of DIILD, in particular the rare variant – tamoxifen-induced OP – allows to identify timely a causal relationship with taking the “responsible” drug and minimize the potential morbidity and risks of adverse evolution of DIILD.

Bronchial asthma is a chronic inflammatory disease of the respiratory tract which requires a multifaceted approach to treatment. The results of modern studies of asthma treatment have shown that combination of beclomethasone dipropionate (BDP)/formoterol fumarate (FF) has a rapid bronchodilating effect, while improving control over asthma symptoms, reducing the frequency of exacerbations, and improving the indicators of external respiratory function. Treatment with BDP/FF is associated fewer side effects compared to systemic corticosteroids (CSs). The rapid onset of action of the fixed BDP/FF combination and the long-lasting effect allow using a single inhaler both for relief of asthma symptoms and for maintenance therapy. The presence of FF in the fixed combination provides rapid bronchodilation, while increasing adherence to basic asthma therapy.

The aim of the study was to evaluate the clinical and pharmacoeconomic advantages of a high-dose fixed combination of extrafine dispersed (EFD) BDP/FF 200/6 μg in patients with asthma in comparison with other combinations of inhaled CSs and long-acting β2-agonists.

Conclusion. EFD BDP/FF at a dose of 200/6 μg can be considered an effective and safe agent for asthma patients whose condition is not adequately controlled with monotherapy with high doses of inhaled corticosteroids (ICSs) or medium doses of ICSs in combination with long-acting β₂-agonists. The clinical advantage of the BDP/FF combination compared to the fixed combination of ICS/salmeterol (SALM) is the significantly faster onset of the bronchodilator effect of FF (within 1 – 3 minutes after the inhalation), along with the increase in the likelihood of asthma control and reduction of the daily consumption of short-acting β₂-agonists compared to the combination of fluticasone propionate/SALM due to the small size of the BDP/ FF molecules.