Primary ciliary dyskinesia (PCD) is a rare hereditary disease from the group of ciliopathies with extensive locus and allelic heterogeneity (ORPHA 244, 98861; OMIM 242650, 244000). This disease is inherited by autosomal dominant or autosomal recessive type and, less often, by X-linked type (OMIM 300424). Retinitis pigmentosa develops in the X-linked PCD variant. The overall minimum global prevalence of PCD according to European data is 1 : 7554. There is no generally accepted classification of PCD in the international classification of diseases (ICD), 10th revision. PCD is not presented in ICD-10 as a separate medical entity, and the code Q32.4 – Other congenital bronchial anomalies – is used for coding. In the new edition of ICD-11, the code LA75.Y is highlighted – Other specified structural abnormalities of the lungs.

Primary ciliary dyskinesia. However, there is no generally accepted classification of PCD. The aim of the study was to develop a classification of primary ciliary dyskinesias to improve the efficiency of medical care for patients during follow-up.

Methods. European and Russian clinical recommendations, as well as ICD 10th and 11th revision, Classification of Respiratory Insufficiency (2020), Order of 27.08.19 No.585n “On classifications and criteria used in the implementation of medical and social expert assessment of citizens by federal state institutions of medical and social expert assessment” (as amended on 06.10.21) were used to create the classification.

Results. The classification of PCD was created and can be recommended for use in clinical practice. The classification was based on the presence or absence of the Sievert – Kartagener syndrome (complete, not complete), as well as clinical and instrumental characterization of bronchopulmonary changes based on the presence of chronic obstructive bronchitis, bronchiectasis (specifying the type and localization), pneumofibrosis with the process activity (exacerbation, remission), and the degree of respiratory failure. It is recommended to take into account extrapulmonary manifestations of PCD, such as rhinosinusitis, media otitis, congenital heart defect, and complications. It is recommended to use the PICADAR (PrImary CiliAry DyskinesiA Rule) score and to include the results of video microscopy, DNA diagnosis, and microbiological examination in the diagnosis.

Conclusion. The application of the proposed classification can be useful in the dynamic observation of the patient, therapy and in the conduct of medical and social expert assessment.

Viral respiratory infection is one of the main etiologic factors for acute exacerbations of COPD (AECOPD). The disease course after virus-associated AECOPD and the response to treatment have not been studied adequately.

The aim was to evaluate the efficacy of single-inhaler triple therapy (SITT) of long-acting anticholinergic drugs (LAMA)/long-acting β₂-agonists (LABA) of adrenergic receptors/inhaled glucocorticosteroids (ICS) on COPD symptoms, lung function, exercise tolerance, and inflammatory activity compared with multiple-inhaler triple therapy (MITT) after a virus-associated COPD exacerbation.

Methods. This was an observational prospective cohort study of COPD patients (spirographус criterion) hospitalized for AECOPD with viral (n = 60) or viral-bacterial (n = 60) infection. The comparison group included patients with bacterial AECOPD (n = 60). Viral infection was diagnosed by PCR-RT of sputum or bronchoalveolar lavage fluid for RNAs of rhinovirus, RS virus, influenza A and B viruses, or SARS-CoV-2 virus. Bacterial infection was demonstrated by sputum purulence and/or procalcitonin test results and/or standard culture data. During AECOPD, blood cytokines were measured by ELISA. The patients were treated with SITT (investigational treatment) or MITT (comparison treatment) of LAMA/LABA/ICS in real-world clinical practice. The follow-up started 4 weeks after AECOPD regression and discharge from hospital.

Results. After 52 weeks of treatment, FEV1 decline was smaller than in the SITT group. Differences from the MITT group were greater in patients with viral AECOPD (–69 (–75; –10) ml vs –75 (–78; –72) ml) or viral-bacterial AECOPD (–67.5 (–69; –37.25) ml vs –75.5 (–84; –70.25) ml). An increase in DL_CO/V_a, 6-minut walk test (6MWT) results, a decrease in bronchodilation coefficient, blood eosinophils and fibrinogen was seen only in subjects who received SITT and had viralor viral-bacterial AECOPD. A multiple regression model revealed a direct association between blood interleukin-5 and improvement in FEV₁ (В = 0.848) and DL_CO/V_a (B=0.117) and a negative correlation with blood levels of amino-terminal propeptide of type-III procollagen and improvement in DL_CO/V_a (В = –0.021).

Conclusion. SITT with LAMA/LABA/ICS was more effective in preventing FEV₁ decline, increasing DL_CO, 6MWT results, and decreasing airway inflammation in virus-associated or viral-bacterial AECOPD.

Major problems in the course of chronic obstructive pulmonary disease (COPD) include frequent exacerbations and related deaths. The use of pneumococcal vaccines, primarily a 13-valent polysaccharide conjugate vaccine, is a generally recognized tool for reducing the number of exacerbations.

The aim of the study was to analyze the prospective clinical efficacy of vaccine prophylaxis with conjugated pneumococcal vaccine Prevenar-13 (PCV13) in patients with COPD over 10 years.

Methods. A total of 362 male patients who were treated or monitored at the Regional Pulmonological Center of Chelyabinsk in 2012 – 2022 were enrolled in the study. The main efficacy endpoints of observation over 10 years were: the dynamics of dyspnea (mMRC score), forced expiratory volume in 1st second, the number of exacerbations, hospitalizations, the number of pneumonias. After confirming the diagnosis of COPD, the patients were divided into two observation groups. The first group (n = 150) included patients vaccinated with PCV13. The second group (n = 212) included patients with COPD who had not received pneumococcal vaccination for various reasons.

Results. Severe forms of the disease and frequent exacerbations predominated in both groups. 96 (27%) deaths were recorded in the PCV13-vaccinated group, and 171 (47%) deaths in the unvaccinated group. The difference between vaccinated and unvaccinated is statistically significant (p < 0.05). In the unvaccinated patients, there was a steady progression of shortness of breath with an increase by 12% compared to baseline (p < 0.05) by the 10^th year. A positive trend in FEV1 was noted in patients vaccinated with PCV13. They also showed a significant decrease in BODE prognostic index.

Conclusion. The use of PCV13 allows for stabilization of the main clinical and functional indicators of the respiratory system in patients with COPD in the long term (at least 10 years of follow-up). Vaccination maintains a low risk of adverse events according to the BODE index; an increase in patient survival was noted over a 10-year horizon (odds ratio – 2.35; 95% confidence interval – 1.45; 3.77), including cases with acute viral damage with COVID-19 (COronaVIrus Disease 2019).

Post-COVID syndrome (PCS) is a pressing problem in clinical practice. Dyspnea, along with neurologic and cardiovascular symptoms, is the most common symptom indicating low exercise capacity (EC) that reduces quality of life. The aim of this study was to investigate shortness of breath and the capillary bed abnormalities detected video capillaroscopy (VCS) in patients with PCS.

Methods. The study included patients with PCS (n = 60) suffering from shortness of breath. Dyspnea was assessed using the 6-minute walk test (6-MWT). The capillary bed abnormality was diagnosed using VCS.

Results. Patients with ACL walked an average of 79.5% of the predicted distance in the 6-MWT. They also had a decrease in pulse oximetry readings. The patients were divided into 2 groups: 1^st – patients with desaturation, 2^nd – patients without desaturation. Significant differences were observed between patients with and without desaturation, especially in the 6-MWT distance, pulse oximetry, and dyspnea before and after the 6-MWT. Patients with desaturation had a higher desaturation-to-distance ratio, a higher O2-GAP index, and higher cardiac effort levels, as well as a significant decrease in the proportion of perfused capillaries (PPC), mean capillary density, and a marked increase in the proportion of tortuous and branched capillaries. There was a direct correlation between PPC and diffusing lung capacity for carbon monoxide (DL_CO), and carbon monoxide transfer coefficient (DL_CO/V_a), distance traveled during the 6-MWT and percentage (%) of predicted distance, inverse correlation between PPC and cardiac effort. There was a direct correlation between the percentage (%) of capillary recovery and 6-MWT distance and the percentage (%) of predicted distance, as well as an inverse correlation with the ratio of desaturation indicators and walking distance. Similarly, there was a direct correlation between mean capillary density and DL_CO/V_a, while the proportion (%) of tortuous capillaries was inversely correlated with DL_CO, and the proportion (%) of branched capillaries was inversely correlated with DL_CO/Va.

Conclusion. The multifactorial nature of the mechanisms of dyspnea in patients with PCS was demonstrated, with the important role of vasculopathy. Continuous monitoring of pulse oximetry, heart rate and VCS are effective methods to screen and assess the condition of patients with PCS and dyspnea.

The purpose of the study using MSCT monitoring data was to investigate the semiotics of residual changes in the lungs in the long-term period (up to 2 years) after pneumonia associated with COVID-19 (COronaVIrus Disease 2019).

Methods. A comparative analysis of MSCT data of the chest organs was carried out in 146 patients with a confirmed diagnosis of COVID-19-associated pneumonia in 2020 – 2021 and subsequent MSCT monitoring data until 2023. MSCT was performed using a 64-slice computed tomography scanner with data post-processed on the workstation.

Results. Based on the analysis, 4 groups of patients were identified that differed in the severity of viral pneumonia and the prevalence of lung lesions. The first group of patients had the most beneficial course. In 90% of cases, there was a complete resolution of the consequences of viral pneumonia in terms macroscopic changes in lung tissue by the second year of observation. In the second group, which consisted primarily of older people, 27 (52.94%) patients in year 1 and in 33 (64.7%) patients in year 2 showed a recovery of macrostructure. This group demonstrated fibrous cords, reticular changes, reactions of pleura, and residual lesions (“ground glass”). A similar trend, but in a more severe form, was observed in group 3 patients. 40 patients (86.95%) had residual changes similar to those in group 2 (middle, elderly age and older people) with the addition of bronchial wall thickening and bronchiectasis. Only in 2 (22.22%) of 9 patients with atypical manifestations of COVID-19 pneumonia in the form of “crazy paving” changes in the form of reticular seals, fibrous cords, localized thickening of the pleura and lymphadenopathy persisted up to 2 years of observation.

Conclusion. MSCT monitoring of the lungs of patients suffering from COVID-19-associated pneumonia up to 2 years after recovery showed that the prevalence and severity of post-COVID changes in the lungs depends on the severity of the disease (degree and nature of damage to the pulmonary parenchyma) and the patient’s age.

Cystic fibrosis (CF) is a multisystem disease of exocrine glands with a progressive course. In recent years, targeted (pathogenetic) treatment aimed at correcting the function of the chloride channel has come to the fore in addition to the symptomatic therapy. The greatest effectiveness was demonstrated with the use of the triple combination of the drug elexacaftor (ELX) / tezacaftor (TEZ) / ivacaftor (IVA) + IVA – Trikafta® (Vertex Pharmaceuticals, USA) that has become the “gold standard” of targeted therapy. Currently, there is virtually no information about the effectiveness and safety of the generic products. The aim of the study was to evaluate the efficacy and safety of the generic drug ELX/TEZ/IVA+IVA (Trilexa®) (Tutor S.A.S.I.F.I.A., Buenos Aires, Argentina) in adult patients with CF in real clinical practice.

Methods. The 6-month study included patients (n = 11) aged 18 to 46 years with a diagnosis of CF who were prescribed pathogenetic treatment with ELX/TEZ/IVA+IVA. Their external respiration function (forced vital capacity, forced expiratory volume in 1 second), conductivity of sweat fluid electrolytes (sweat test), anthropometric and other clinical and functional data were analyzed. Results. This study demonstrated pronounced positive effects in relation to indicators of respiratory function, sweat test, and nutritional status. The ELX/TEZ/IVA+IVA treatment was well tolerated, with clinical improvement in the form of a decrease in cough intensity, sputum volume, improvement in daily exercise tolerance, height, and body weight. No serious adverse events were recorded and none of the patients discontinued treatment due to adverse reactions.

Conclusion. Obvious clinical and functional positive dynamics and safety over 6 months were demonstrated with ELX/TEZ/IVA+IVA combination.

The development of new methods for diagnosing infectious diseases, such as polymerase chain reaction, sequencing, and mass spectrometry, has made it possible to expand our understanding of the microflora of the human body in general and of the respiratory tract in particular. The most common microorganisms in lungs include Proteobacteria (Pseudomonas spp., Haemophilus spp.), Bacteriodetes (Prevotella spp., Porphyromonas spp.) and Firmicutes (Veillonella spp., Streptococcus spp.). Pathological processes in the lower respiratory tract change the microbiome. Consequently, Moraxella, Haemophilus, and Acinetobacter microorganisms are found in patients with chronic obstructive pulmonary disease. The main causative agents of community-acquired pneumonia include Streptococcus pneumoniae, Staphylococcus aureus, Haemophilus influenzae, Klebsiella pneumoniae and other enterobacteria. Atypical causative agents of pneumonia include Mycoplasma pneumoniae, Chlamydia pneumoniae, and Legionella pneumophila. The wide prevalence of S. pneumoniae in the population confirms the need for vaccines to prevent the development of invasive and noninvasive forms of infection.

The aim of the study was to analyze literature data on the structure of the normal microflora of the lung and to investigate its role in the development of pathological conditions.

Conclusion. A growing body of research confirms the important role of the normal microflora of the respiratory tract. Vaccination against diseases of the bronchopulmonary system may reduce the incidence and mortality of pneumonia. However, the problem of community-acquired pneumonia is still relevant. In particular, the changes in the structure of the pathogens of this disease is of great interest. Vaccination against respiratory pathogens in combination with uncontrolled use of antibiotics during the pandemic of the new coronavirus infection could contribute to a change in the structure of both the lower respiratory microflora in general and the pathogens of bacterial community-acquired pneumonia in particular. The detection of a change in the predominant pathogen calls for revising etiotropic treatment and organizing planned prophylaxis if the appropriate vaccines are available.

Oscillometry (the forced oscillation technique) is widely used in the Russian Federation. It is a simple, noninvasive functional diagnostic test that offers unique advantages in cases where spirometry and other pulmonary function tests are not appropriate, such as in young children, debilitated patients, or patients with physical and cognitive barriers to performing tests that require effort and coordination.

The aim of this study was to analyze scientific literature data and our own results and to evaluate the significance and applications of oscillometry in clinical practice. The review examined the physiological principles of oscillometry and the basis for interpreting the results. Oscillometry is now recognized as a sensitive diagnostic tool for assessing airway resistance. This article describes sensitivity of the method for bronchodilation and bronchoprovocation tests, as well as its response to drug treatment, especially in asthma and chronic obstructive pulmonary disease. Oscillometry is a useful tool for diagnosing obstructive ventilation disorders. It should be used as an addition to spirometry to clarify phenotypes of small airways diseases. However, despite its great promise as a useful diagnostic tool, further evidence of its clinical utility in restrictive conditions is needed.

Conclusion. Thus, oscillometry can be considered an addition and sometimes an alternative to spirometry and bronchodilation test, since it is well standardized. The test results are highly reproducible, the study does not require maximum effort, and the test is carried out during spontaneous tidal breathing.

The aim of this review was to analyze current information on tiotropium bromide as one of the LAMAs. Tiotropium was chronologically the first LAMA in clinical practice. It is administered using a dry powder inhaler or an aerosol inhaler (Respimat soft mist inhaler). Due to the long bronchodilator effect, tiotropium can be inhaled once a day. The drug has a significant effect on functional respiratory parameters, reduces lung hyperinflation, improves exercise tolerance and quality of life. Tiotropium is superior to short-acting bronchodilators and long-acting β₂-agonists (LABAs) and is comparable to other LAMAs, as well as combinations of LABAs and inhaled corticosteroids in the prevention of COPD exacerbations. Tiotropium bromide has a favorable safety profile. Generic tiotropium drug products, including Respium®, are currently available in the Russian Federation. A clinical trial confirmed the non-inferiority and safety of generic tiotropium drug compared to the original branded product.

Conclusion. Tiotropium is an effective and safe medication for the maintenance treatment of COPD and for reducing COPD exacerbations. The generic tiotropium drug products widen the range of effective inhaled medications available to physicians.

Pulmonary hemorrhage is a serious complication of cystic fibrosis (CF) with a prevalence up to 9.1%. According to the Registry of Patients with Cystic Fibrosis of the Russian Federation for 2020, the prevalence of pulmonary hemorrhage was 1.5% in the general population, and 6.5% in patients over 18 years of age. Despite the importance of this problem, there is no systematic information on pulmonary hemorrhage in CF.

Aim. The purpose of this study was to review current data on the pathogenesis, risk factors, and approaches to the treatment of pulmonary hemorrhage in patients with CF. Information was searched in еLibrary and PubMed databases.

Results. During the literature review, we summarized and systematized the collected scientific data on pulmonary hemorrhage in CF. The pathogenesis of pulmonary hemorrhage is chronic inflammation in the bronchial wall accompanied by increased angiogenesis factors that promote neovascularization with development of many tortuous, thin-walled vessels. Exacerbation of bronchial inflammation and mechanical strain contribute to erosion and damage of fragile vessels and cause the bleeding. The main risk factors for pulmonary hemorrhage were colonization with Pseudomonas aeruginosa, Staphylococcus aureus, diabetes mellitus, and FEV1 < 70%. Complex therapy for pulmonary hemorrhage includes the use of antibiotics and antifibrinolytic agents. Some of the published regimens use β-blockers and blood coagulation factor VII. Data on the efficacy of vitamin K are unclear, but it is also used in the treatment of pulmonary hemorrhage. In cases of massive bleeding, temporarily discontinuation of inhalations of hypertonic solution and kinesiotherapy is recommended. In case of recurrent massive bleedings, bronchial artery embolization is performed and lung resection can be done in extreme cases.

Conclusion. Hemoptysis in CF is a complex clinical problem that requires a multidisciplinary approach. Currently, important aspects of the pathogenesis of pulmonary hemorrhage remain unclear. Given the increasing life expectancy of CF patients and the age-related risk of pulmonary hemorrhage, it is important to investigate the pathogenetic aspects of this complication for the subsequent development of effective and justified treatment algorithms.

Thymic neoplasms account for 20 – 25% of primary mediastinal tumors. Nonspecific clinical symptoms are often associated with delayed diagnosis of thymoma, leading to a distinctive problem of late diagnosis. Nowadays, surgical removal of the tumor is the method of choice for thymoma treatment. Combination treatment is indicated in the case of an unresectable tumor. Modern therapy (various combinations of anticancer drugs) helps reduce neoplasm size, stop metastasis, and maintain good quality of life in patients with thymoma.

Aim. The presented clinical case of type B3 thymoma (with invasion into surrounding organs and metastatic pleura) is characterized by a long asymptomatic period of the disease, the onset of symptoms after suffering COVID-19 (COronaVIrus Disease 2019) and successful long-term polychemotherapy. The causes of late diagnosis, as well as insufficient oncologic vigilance in evaluating the results of laboratory and instrumental tests, are discussed based on this clinical case.

Conclusion. A correct diagnosis, as well as successful treatment, requires a multidisciplinary approach involving physicians from different specialties – general practitioners, oncologists, surgeons, radiologists, and morphologists.

Diagnosis of interstitial lung diseases (ILD) in individuals infected with the human immunodeficiency virus (HIV) remains poorly understood.

The purpose of the work was to demonstrate approaches to the diagnosis of ILD in HIV-infected patients with different levels of CD4 lymphocytes. An analysis of literature data and clinical observations of ILD in HIV-infected individuals (n = 2) whose CD4 lymphocyte count was 677 (37.5%) and 1 (0.21%) cells/μl are presented. The patients were observed and treated for a long time by pulmonology specialists, including anti-tuberculosis chemotherapy. The patient with CD4 lymphocyte count of 677 cells/μl in the absence of Mycobacterium tuberculosis in the sputum had an established diagnosis of idiopathic pulmonary fibrosis with the progressive development of respiratory failure and progression of the computed tomographic pattern from large areas of “ground glass” to the formation of reticular changes and “honeycomb” lung within 2 years. The patient with CD4 count of 1 cell/μl was diagnosed with nonspecific interstitial pneumonia based on characteristic clinical manifestations of increasing respiratory failure against the background of diffuse interstitial pneumofibrosis in the absence of bacterial excretion, the effects of antimycobacterial, antibacterial and antifungal treatment for 7 months, and excluding other causes of lung damage.

Conclusion. The development of interstitial fibrosing pulmonary process of non-infectious etiology in HIV-infected patients with different states of T-helper immunity has been demonstrated. After excluding opportunistic infections, the influence of other factors, and concomitant diseases, these patients were diagnosed with various forms of idiopathic interstitial pneumonia. However, existing reports of the direct damaging effects of the virus on the pulmonary parenchyma also suggest the possibility of HIV-associated interstitial pulmonary fibrosis.

Relevance. Tuberous sclerosis complex (TSC) is a rare genetically determined disease from the group of phakomatoses, which is characterized by a wide range of damage to organs and systems, a variety of clinical manifestations and a progressive course, leading to disability of patients and a reduction in life expectancy. Benign tumors (hamartomas) can occur with TSC in various organs, including the brain, eyes, skin, heart, kidneys, liver, lungs, gastrointestinal tract, endocrine, and skeletal systems. Gradually progressing and increasing in size, they disrupt the functions of these organs, sometimes leading to fatal consequences. Objective. Demonstration of clinical observation of TSC, the main manifestation of which was lung damage. Conclusion. The presented clinical case demonstrates the difficulty of diagnosing TSC, the features of its relatively benign course.

Common concomitant endocrine diseases in chronic obstructive pulmonary disease (COPD) patients include both structural and functional lesions of the thyroid gland.

The purpose of the study was to determine specific indicators for the differential diagnosis of thyroid storm and exacerbation of COPD. A clinical observation of a 60-year-old patient who received inpatient treatment in the therapeutic department of a city clinical hospital is presented. The diagnosis upon admission was COPD, mixed form, severe, exacerbation, and respiratory failure of the first degree.

Results. In the hospital, the patient experienced an episode of difficulty breathing lasting 45 minutes. Increased shortness of breath alternated with normal breathing and was accompanied by tremor of the hands. The patient was agitated, refused to breathe oxygen through the mask, and was in orthopnea. The patient’s condition during the attack was assessed at 40 points according to the Burch – Wartofsky scale, which corresponds to a high probability of thyroid storm (TS). Pronounced ventilation disturbances of a mixed type were recorded based on the spirography results (forced expiratory volume in 1 second – 19%). Echocardiography revealed no signs of cor pulmonale or pulmonary hypertension. A low concentration of thyroid-stimulating hormone was recorded, the level of free thyroxine (T4) remained within the reference range. Serum levels of thyroid peroxidase antibodies did not increase, and no changes in the thyroid gland were detected during ultrasound examination.

Conclusion. When thyroid abnormality manifests in a patient with COPD, certain difficulties may arise with the differential diagnosis of exacerbation of COPD and TS due to the similarity of their clinical manifestations.