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PULMONOLOGIYA

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Vol 36, No 1 (2026)
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EDITORIAL

7-17 375
Abstract

The metabolic and immunological functions of the lungs are the subject of active scientific research. In recent years, methods for studying organic and inorganic volatile compounds in exhaled air and its condensate have been standardized. The metabolic function of the lung parenchyma involves the regulation of water and electrolyte balance, as well as biologically active amines and peptides. The unique properties of type 2 alveolar cells and alveolar macrophages are manifested in the synthesis and recirculation of surfactant. Pulmonary capillary endothelial cells play a special role, converting angiotensin-1 (a decapeptide) into angiotensin-2 (an octapeptide) with the help of angiotensin-converting enzyme.

The aim was to study the metabolic, immunological and endocrinological functions of the lungs, as well as the diagnostic role of exhaled air condensate.

Results. The adult lungs contain approximately 20% immune cells, which maintain normal homeostasis and participate in the implementation of a protective barrier against various pathogens and allergens. A significant role is played by mucosal immunity, which involves mucosa-associated lymphoid tissue, immune cells, secretory immunoglobulin A, and various enzymes. Clinical manifestations of immunopathological reactions of the respiratory system are diverse. Thus, allergic reactions of both immediate and delayed types can be observed in clinical practice. Granulomatous processes of the pulmonary parenchyma have a special place. Neuroepithelium in the mucous membrane of the respiratory tract plays an important role in the differentiation of cell structures and in the morphogenesis of lung tissue. However, the participation of the lungs in endocrinological reactions remains a poorly studied direction.

Conclusion. The research of metabolic, immunological and endocrinological functions of the lungs identified a number of biological markers associated with bronchial asthma, COPD, lung cancer, and pneumonia. Studies of the proteome of exhaled air condensate have brought us closer to the discovery of new biological markers.

ORIGINAL STUDIES

18-30 582
Abstract

Uncontrolled asthma poses a widespread problem in Russia, fueled by the overuse of short-acting β2-agonists (SABA).

The aim of the study titled “Clinical and demographic profiles of patients with uncontrolled asthma in Russia: multi-center observational registry study (ICEBERG)” is to investigate the clinical and demographic profile and treatment approaches in this patient population.

Methods. An interim analysis of data from 2,024 adult patients with mild to moderate uncontrolled asthma was conducted. This multicenter observational retrospective-prospective study included two visits: baseline (collecting 52-week retrospective data and current status) and a 12-week follow-up visit to assess changes in therapy and clinical outcomes. Asthma control was assessed using Global Initiative for Asthma (GINA) criteria and the Asthma Control Questionnaire-5 (ACQ-5).

Results. Patients with uncontrolled asthma predominantly exhibited a T2-high phenotype. A marked shift from SABA therapy to controller regimens was observed following the confirmation of uncontrolled disease: the proportion of patients on MART doubled (from 9.5% to 20.4%). This treatment optimization led to improved asthma control (full or partial control achieved in 70.6% of patients) and a significant reduction in the frequency and duration of exacerbations. The use of SABA as primary therapy remained a key risk factor for severe exacerbations.

Conclusion. The consistent implementation of GINA strategies, in particular the transition to fixed-dose ICS/SABA combination therapy, the use of the MART regimen, and triple therapy, makes it possible to significantly improve the asthma control and reduce the risk of exacerbations in real-world clinical practice.

31-42 318
Abstract

Interstitial lung diseases (ILD) represent a serious medical and social problem due to their progressive course and unfavorable prognosis. Genetic factors, in particular, the MUC5B gene polymorphism, play an important role in their pathogenesis.

The aim of the study was to evaluate the frequency of the T-risk allele of the rs35705950 single nucleotide polymorphism of the MUC5B gene in Russian patients with hypersensitivity pneumonitis (HP) and idiopathic pulmonary fibrosis (IPF) compared with the general population.

Methods. The study included patients (n = 86) with HP (n = 49) and IPF (n = 37). The control group consisted of a sample from the National Genetic Initiative 100,000 + I project (n = 89,261). Genotyping of the rs35705950 polymorphism was performed using polymerase chain reaction followed by restriction fragment length polymorphism analysis (BstHHI restriction enzyme). Statistical analysis included calculation of allele and genotype frequencies, assessment of compliance with Hardy – Weinberg equilibrium, and association analysis based on odds ratios (OR) for allelic and dominant models.

Results. The frequency of the risk allele T was significantly higher in the patient groups compared to the control (p < 0.001): 0.2973 in IPF (OR – 3.82; 95% CI – 2.21–6.41; p =  1.9 × 10–6) and 0.2551 in GP (OR – 3.10; 95% CI – 1.88–4.94; p = 8.4 × 10–6). The results of the dominant model analysis (TT+GT genotypes) also revealed a significant increase in the risk of IPF (OR – 5.05; 95% CI – 2.64–9.64) and GP (OR – 3.22; 95% CI – 1.83–5.67) compared with the control group (p < 0.001). When comparing patients with IPF and GP, the differences in T allele frequencies did not reach statistical significance (OR – 1.23; 95% CI – 0.59–2.56; p = 0.61). The distribution of genotypes in all groups corresponded to the Hardy – Weinberg equilibrium.

Conclusion. The rs35705950 polymorphism of the MUC5B gene is a significant genetic risk factor for the development of both IPF and GP in the Russian population. The similar risk profile for these two entities suggests common pathogenetic mechanisms associated with this gene. These findings support the inclusion of MUC5B analysis in the evaluation of patients with suspected ILD.

43-51 236
Abstract

One of the most important predictors of the prognosis of cystic fibrosis (CF) is the pulmonary function test – forced expiratory volume in 1 second (FEV1). It is important for physicians to understand the factors associated with changes in FEV1 in patients not receiving CFTR modulators.

The aim of this study was to identify factors influencing changes in FEV1 in patients with CF not receiving CFTR modulators.

Methods. Retrospective analysis was conducted using medical records of patients (n = 225) under 18 years who received inpatient treatment in the pediatric department of the Russian Children’s Clinical Hospital, the Branch of the Russian National Research Medical University named after N.I.Pirogov, as well as patients over 18 years (n = 181) who were followed by specialists of the Pulmonology Scientific Research Institute under Federal Medical and Biological Agency of Russian Federation.

Results. Nutritional status and the nature of pathogenic microflora of the lower respiratory tract have a key impact on the FEV1 indicator in childhood. In the group of adult patients, the leading role belongs to complications such as CF-associated diabetes mellitus and the presence of pneumothorax.

Conclusion. Regular monitoring of external respiratory function is an integral part of patient management in CF. This study demonstrates the relationship between FEV1 and a number of factors in patients not receiving pathogenetic therapy.

52-64 352
Abstract

COVID-19 (COronaVIrus Disease 2019) remains a significant challenge, as patients continue to experience long-term cardiopulmonary complications such as interstitial lung changes (ILCs)/pulmonary parenchyma (LP) and pulmonary hypertension (PH) after acute coronavirus infection.

The aim of this study was to identify clinical and functional indicators for detecting pulmonary hypertension (PH) in patients with ILCs during the long-term follow-up after acute COVID-19.

Methods. The study included patients (n = 75) with post-COVID-19 pneumonia who developed ILI after the event and were hospitalized at the University Clinical Hospital No.4 at Sechenov University (Federal State Autonomous Educational Institution of Higher Education I.M.Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation), from March 2022 to February 2024, 17 (8; 24) months after COVID-19. A multidisciplinary team defined the lung condition as ILCs associated with past coronavirus pneumonia. Based on echocardiography (EchoCG) results, patients were divided into two groups: group 1 (n = 45) with PH (pulmonary artery systolic pressure (PAS) ≥ 35 mmHg) and without PH (PAS < 35 mmHg) (n = 30).

Results. Based on EchoCG data, the groups differed in right heart parameters and in the extent of lung parenchyma (LP) damage, as determined by computed tomography (CT) scans during the acute phase of COVID-19. The majority of patients in the PH group had LP damage stage 4 (CT-4) (p < 0.05) according to CT scans; however, no differences in lesion extent were found based on CT scans at the time of follow-up. Lower values of the diffusing capacity of the lungs for carbon monoxide (DLCO) were found in the PH group compared to the control group (p < 0.05). ROC analysis demonstrated the diagnostic capabilities of DLCO in detecting PH: the sensitivity and specificity of the method were 77.8% and 62.5%, respectively, at a threshold of 70% of the predicted value (p = 0.006). Based on the results of univariate logistic regression analysis, significant predictors of the presence of PH were identified. A 1% decrease in DLCO was associated with an increased chance of developing PH (odds ratio (OR) – 0.973; 95% confidence interval (CI) – 0.951 – 0.995; p = 0.016). The probability of PH with desaturation during exercise increased 4.2-fold (OR – 4.229; 95% CI –1.570 – 11.394; p = 0.004). An increase in the Borg scale score by 1 point after the 6-minute walk test (6-MWT) was associated with a 49% increase in the odds of PH (OR – 1.489; 95% CI – 1.117 – 1.984; p = 0.007). A higher stage of lung damage according to CT in the acute period was also a significant predictor (OR – 1.619; 95% CI – 1.045 – 2.509; p = 0.031). In the multivariate model, a DLCO decrease of <70% remained an independent predictor of PH (adjusted OR – 4.39; 95% CI – 1.11 – 17.40; p = 0.035).

Conclusion. Patients with ILCs associated with previous coronavirus infection are characterized by a DLCO decrease of < 70% and impaired exercise tolerance. The identified threshold values of functional parameters can be used for screening for PH, which justifies the need for dynamic monitoring of patients in this category.

65-70 288
Abstract

Background and Objectives: Pleural effusion is defined as an abnormal accumulation of fluid in the pleural space. The diagnostic approach to pleural effusions depends on the clinical context and initial pleural fluid aspiration. However, in some cases, pleural fluid analysis fails to establish a definitive diagnosis. This study aims to evaluate the diagnostic value of medical thoracoscopy in patients with unexplained exudative pleural effusions.

Material and Methods: A cross-sectional study was conducted at the respiratory unit of Al Dowaly Private Hospital, Baghdad, Iraq, over a period of 9 months. Twenty-five patients with unexplained exudative pleural effusions, after non-diagnostic pleural fluid analysis, were included. Patients with hemodynamic instability, coagulopathy, serological or PCR evidence of current COVID-19 infection, or a previously established diagnosis of pleural effusion were excluded.

Results: Medical thoracoscopy provided a definitive diagnosis in 19 out of 25 patients (76%). Among these, malignancy was identified in 64% of cases, while tuberculosis accounted for the remaining diagnoses.

Conclusion: Medical thoracoscopy is a valuable diagnostic tool with high yield and minimal complications. It is recommended for patients with undiagnosed exudative pleural effusions following inconclusive initial work-up.

 

REVIEW

71-83 246
Abstract

Asthma is a chronic disease characterized by chronic inflammation, hyperresponsiveness, and reversible obstruction of the lower respiratory tract (RT). Airway muscles (ASM) play a central role in the pathophysiology of asthma, being essential for the narrowing and remodeling of the RT. Local hyperthermia of the ASM, achieved by bronchial thermoplasty (BT) and inhalation of a heated helium-oxygen mixture (thermal heliox, t-He/O2), reduces the ASM mass and contractile activity, which may have a positive pathogenetic effect on the symptoms and course of asthma.

The aim of this review is to study the relationship between the temperature of the ASM and the functional state in patients with asthma, as well as the therapeutic effect of thermotherapy in this disease based on an analysis of the literature from PubMed and Google Scholar.

Methods. 85 publications in PubMed and Google Scholar were selected by searching the following key words: “ASM”, “contraction of ASM”, “relaxation of ASM”, “hyperreactivity of the airways”, “chronic inflammation”, “asthma”, “hyperthermia of the airways”, “bronchial thermoplasty”, “thermal helium-oxygen mixture” (t-He/O2).

Results. Based on literature data, an assessment was made of the clinical efficacy and safety of two methods of thermal modulation of ASM: BT and inhalation of t-He/O2. Local hyperthermia of the ASM, achieved by BT and inhalation of a t-He/O2 reduces the mass and contractile activity of ASM, which may have a positive pathogenetic effect on the symptoms and further course of BA.

Conclusion. Studying the role of temperature in maintaining local inflammation and the development of bronchial obstruction syndrome is not only of great scientific importance, but also has unquestionable practical value for the development of new promising methods of treating asthma.

84-94 357
Abstract

Recent studies demonstrate that the combination of asthma and bronchiectasis is a common phenomenon, leading to a more severe course of asthma with frequent exacerbations. The mechanisms underlying the association between these two diseases are not well understood; however, they share common clinical and immunological features, as well as numerous comorbidities.

The aim of this review was to examine and analyze the results of studies focusing on concomitant asthma and bronchiectasis.

Methods. A literature search in the PubMed, MedLine databases was conducted using the following key terms: bronchiectasis in asthma, association between asthma and bronchiectasis, comorbidities in asthma, severe asthma, treatment of asthma and bronchiectasis. The publication date was not limited.

Results. Asthma and bronchiectasis share overlapping symptoms, similar comorbid conditions, and etiopathogenetic mechanisms. The coexistence of these two diseases may form a characteristic phenotype with a more severe course of asthma. The “asthma + bronchiectasis” phenotype is characterized by more severe bronchial obstruction, frequent exacerbations, and longer hospitalizations as compared to asthma alone. Patients with asthma and chronic rhinosinusitis have an especially high risk of developing bronchiectasis. Recently, bronchiectasis has been viewed as an inflammatory, rather than purely infectious, disease, highlighting the complexity of its pathophysiology. Bronchiectasis with high eosinophil levels is of particular interest. The “asthma + bronchiectasis” phenotype reflects the overlap of eosinophilic and neutrophilic inflammatory mechanisms, leading to airway remodeling, while microbiota dysbiosis exacerbates this inflammation. This creates a vicious cycle of airway inflammation and damage. Early detection of bronchiectasis with high-resolution computed tomography in patients with severe asthma is essential for initiating appropriate therapy for both diseases. This approach improves symptom control and reduces the frequency of exacerbations and infections.

Conclusion. Bronchiectasis is a common comorbid condition in patients with asthma. The combination of these two diseases constitutes a distinct phenotype characterized by more frequent chronic infections and increased disease severity. Patients with the “asthma + bronchiectasis” phenotype require an individualized approach to optimize treatment and effectively address this complex comorbidity.

EXPERT OPINION

95-106 693
Abstract

Chronic obstructive pulmonary disease (COPD) is a common respiratory disease, a leading cause of morbidity and mortality worldwide. In recent years, new approaches to the epidemiology, diagnosis, classification (categorization), phenotype assessment, and characterization and severity of exacerbations of COPD have emerged. Modern approaches to initial and subsequent drug therapy have changed significantly. This is largely due to the results of large studies conducted in recent years, demonstrating the high efficacy of triple fixed-dose combinations including inhaled glucocorticosteroids, long-acting β-agonists, and long-acting anticholinergics.

The aim  of this review was to highlight the latest advances in COPD treatment, taking into account the nuances of the pathophysiology of its subtypes, which will be crucial for reducing the symptoms of the disease. Due to the emergence of new drugs, there was a need to modify domestic algorithms for managing patients with COPD.

Methods. For the first time, the biological agent dupilumab has been included in clinical guidelines; it is indicated for patients with COPD with signs of type 2 inflammation and symptoms of chronic bronchitis. The use of non-pharmacological therapies (smoking cessation, physical activity, and respiratory rehabilitation) and modern approaches to treating respiratory failure remain important.

Conclusion. New additions to clinical guidelines emphasize the importance of early intervention, personalized treatment, and the diversity of COPD phenotypes, and also lay the foundation for a more effective and individualized COPD treatment model. The ability to control inflammation and improve lung function in COPD patients using modern therapeutic approaches can not only improve quality of life for patients but also potentially reduce the overall burden on the healthcare system.

PRACTICAL NOTES

107-117 986
Abstract

Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are hereditary lung diseases. Cystic fibrosis is a monogenic disease, while PCD has a genetically heterogeneous nature and is 2 times less common than CF. More than 50 genes are responsible for PCD, one of them being the DNAAF11 gene (LRRC6 according to the previous nomenclature) – the assembly factor of the external and internal dynein arms. When the mutation is present, components of the dynein arms remain in the cytoplasm of cells.

The aim is to present a clinical case of a newly diagnosed PCD in a 44-year-old woman with a homozygous pathogenic variant of the nucleotide sequence NM_012472.6:c.436G>C in the DNAAF11 gene. This clinical case demonstrates the complexity of verification and differential diagnosis of cystic fibrosis and primary ciliary dyskinesia, the features of the disease, and the modern diagnostic capabilities. The difficulties and some typical mistakes in the management of such patients are presented.

Conclusion. The presented clinical case illustrates the difficulties of PCD diagnosis. It demonstrates that such patients need a comprehensive examination for a thorough differential diagnosis to exclude diseases with a similar clinical picture. The long-term follow-up is carried out by a multidisciplinary team of specialists and must include microbiological monitoring.

118-122 248
Abstract

Pulmonary sequestration (PS) is a developmental anomaly of lung tissue in which the abnormally developed section is disconnected from the normal bronchial system and is supplied by branches of aberrant vessels. Clinical manifestations of this defect typically begin in childhood, but diagnosing PS in adults is also possible. Visualization of the aberrant vessel supplying the pathological section is of important diagnostic value.

The aim of this study was to demonstrate the feasibility of diagnosing late-onset PS.

Conclusion. The presented clinical observation highlights the complexities and sequence of diagnostic and differential diagnosis steps for a rare pulmonary anomaly presenting as community-acquired pneumonia.

123-132 339
Abstract

The incidence of dirofilariasis in humans is increasing worldwide. Dirofilariasis is the only mosquito-borne transmissible helminthiasis recorded in the Russian Federation. The definitive hosts of this helminth are dogs, cats, and wild predators; humans are rarely infected. The most common infections in humans and animals are caused by Dirofilaria repens and Dirofilaria immitis. The main sites of dirofilaria infestation in humans are the subcutaneous tissue, periorbital area, and lungs. Pulmonary dirofilariasis is usually asymptomatic, detected as a focal or pathological lesion on chest X-ray. Currently, noninvasive laboratory diagnostic methods for dirofilariasis are lacking; diagnosis is established by histological examination of lung tissue. Treatment of the disease is usually surgical.

The aim of the work was to demonstrate and discuss in detail the epidemiology, clinical picture, approaches to diagnosis and treatment of two clinical observations of the classic low-symptom course of pulmonary dirofilariasis. Surgical intervention was necessary to exclude a malignant lung tumor. The epidemiological features, clinical, radiological, and pathological presentation of the disease, complications, and patient management are discussed.

Conclusion. Pulmonary dirofilariasis should be considered in the differential diagnosis of focal and pathological lung lesions.

Improvement of noninvasive laboratory methods for detecting this helminthiasis in humans is necessary.

133-141 261
Abstract

Echinococcosis and alveococcosis of the lungs are zoonotic infections caused by the larval stage of Echinococcus helminths. The difficulty of diagnosing these helminth infections is due to their slow development and their similarity to several diseases, including malignant neoplasms and tuberculosis. Delay in making the correct diagnosis leads to the development of advanced cases and longer treatment.

The aim of the review was to inform physiatrists, pulmonologists, infectious disease specialists, therapists, and thoracic surgeons about the complexity of the differential diagnosis of echinococcosis with atypical limited localization (with isolated lung damage).

Conclusion. Diagnosis of echinococcosis presents significant difficulties due to the absence of symptoms pathognomonic for the parasitic process and a typical clinical manifestation of the disease, due to similarity with a number of nosologies of infectious and non-infectious origin, and absence of liver lesions and multilocular cysts, usually detected by CT or MRI. An immunological blood test for specific IgG should be performed at the early stages of diagnosis in patients without bacterial excretion, with suspected tuberculosis, and with an ambiguous clinical picture of pulmonary lesions. However, sometimes this blood test can be false negative because of a weak immune response.

142-146 291
Abstract

Sarcoidosis is a chronic polysystemic disease of unknown etiology, classified as granulomatosis. Atypical radiological manifestations of the disease may challenge timely diagnosis.

The aim of this work was to present a clinical case demonstrating the capabilities of modern diagnostic methods for disseminated lung diseases. The article presents a clinical case of a 39-year-old patient who was registered at a phthisiological clinic and received anti-tuberculosis therapy at a tuberculosis dispensary. The patient was referred to the Federal State Budgetary Institution “Novosibirsk Tuberculosis Research Institute” of the Ministry of Health of the Russian Federation, where cryobiopsy and pathomorphological examination led to diagnosing sarcoidosis.

Conclusion. Modern high-tech endoscopic equipment allows for more informative investigations and shorter time to correct diagnosis. 

LETTER TO EDITOR



ISSN 0869-0189 (Print)
ISSN 2541-9617 (Online)