Objective: to evaluate treatment effectiveness in patients with mycobacterial pulmonary disease and its association with mycobacteria detection in sputum. Materials and methods. The study enrolled 196 patients with confirmed mycobacterial pulmonary disease. The diagnosis was established as per ATS/IDSA criteria (2007). The patients were divided into two groups: group 1 consisted of 108 patients with nontuberculous mycobacteria (NTM) detected in sputum; group 2 consisted of 60 patients with NTM detected in BAL and/or lung tissue samples. Female patients prevailed (78.6%). The average patients’ age was 56.9 ± 11.3 years. The time period from diagnosing pulmonary mycobacterial disease to commencementof combination antibacterial therapy varied from one month to 3.5 years. Patients were followed up during one year after treatment completion. Results. The most commonly identified NTM were MAC (M. аvium, M. intracellulare) and M. kansasii. The combination antibacterial therapy was administered to 168 (85.7%) patients. Clinical cure was more frequently achieved in the patients of group 2 compared to group 1 (21.7 and 3.7% respectively, р < 0.01). The frequency of clinical improvement without conversion of biological samples did not significantly differ between the groups (40 and 54.6% respectively, …). The frequency of relapse was similar in both groups and did not exceed 5%. Conclusion. The presence of NTM in BAL while the absence of NTM in sputum allows predicting more favourable disease course and better response to treatment.

The pulmonary disease caused by nontuberculous mycobacteria (NTM) is a chronic respiratory infection resulting in declining lung function, worsening life quality, and increasing mortality rates. Most patients with pulmonary nontuberculous mycobacteriosis (PNTM) have concomitant chronic diseases (bronchiectatic disease, COPD, diabetes mellitus, etc.) PNTM is a refractory disease as mycobacteria are located intracellularly in alveolar macrophages. Mycobacteria can develop a biofilm, which impedes antibiotic penetration and protects them. Treatment for PNTM is long-term with low effectiveness due to innate or acquired mycobacterial resistance. The article outlines the modern concepts of drug resistance development in NTM, refractoriness of the disease, and the current mechanisms to overcome it.

Sarcoidosis is a multi-organ granulomatosis of unknown origin. Modern diagnostic methods allow detecting this disease at an early stage. The absence of specific markers requires a comprehensive approach to diagnosis based on comparison of radiation, clinical, morphological and functional data. The course of sarcoidosis without damage to the respiratory system presents significant difficulties. It is extremely important to understand the time and means of starting Sarcoidosis’s treatment to avoid the early initiation of hormones and cytostatics and, on the other hand, to timely respond to progression and threatening conditions. Methods. Clinical recommendations are based on the analysis of Russian and English publications of the latest sarcoidosis research. The target audience of these clinical guidelines are therapists, general practitioners, pulmonologists, TB doctors, rheumatologists, dermatologists, radiation diagnosticians, immunologists, and clinical pharmacologists. Each thesis-recommendation for diagnosis and treatment is evaluated on an 1 to 5 scale of levels of evidence and an A, B, C scale of the grades of recommendations. The clinical guidelines also contain comments and explanations for the theses-recommendations, diagnostic algorithms, treatment strategies, reference materials on the use of recommended drugs. Conclusion. Current information on epidemiology, clinical manifestations, diagnosis and management strategies for patients with sarcoidosis are covered in the presented clinical guidelines. Approved by the decision of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (2022).

Objective: to describe the structure of nosological forms of pulmonary diseases in differential diagnosis of lung cavities in a federal TB institution and assess the effectiveness of diagnosis verification based on studies of different samples (sputum, bronchoscopic samples, resection samples). Materials and methods. We conducted a cohort prospective open study. We examined 122 patients with newly detected lung cavities at the Central TB Research Institute: 60 (49%) women and 62 (51%) men with the average age of 42.9 ± 14.2 years. Results. Among 122 patients with lung cavities the following diagnoses were verified: 41.8% of pulmonary TB cases, 41% of pulmonary mycobacteriosis cases, 11.5% of destructive pneumonia cases, and 5.7% of lung cancer cases. Destructive TB was verified based on: complex sputum study – 54.9%, complex study of bronchoscopic samples – 91.3% (р = 0.003), and complex study of resection samples – 100% of cases (р = 0.016). The study of bronchoscopic samples was the most significant for the verification of destructive pneumonia – 100% vs. 28.6% of cases verified by sputum study (р < 0.001, χ2 = 12.245). Pulmonary mycobacteriosis was verified based on sputum study in 28% of cases vs. 44.4% of cases verified by bronchoscopic sample study, though without statistically significant difference (р = 0.223, χ2 = 1.490); only resection sample study enabled to verify the diagnosis in 100% of cases (р < 0.001, χ2 = 17.959). Among patients with pulmonary mycobacteriosis, slowly growing nontuberculous mycobacteria (NTM) were isolated in 92% of cases; the most common were M. avium complex (46%) and M. kansasii (28%). Lung cancer was confirmed by bronchoscopic study in 57.1% of cases, while resection sample study confirmed the diagnosis in 100% of cases. In outpatient settings the verification of lung cavity differential diagnosis based on sputum or bronchoscopic sample study was effective in 79.5% of cases. Hospitalization for surgical diagnostic procedures was required in 20.5% of cases. Conclusion. In the structure of nosological forms of lung cavities pulmonary TB (41.8%) and pulmonary mycobacteriosis (41%) prevailed. We established high effectiveness of diagnosis verification in lung cavity patients based on complex study of bronchoscopic samples or resection samples performed in the federal TB centre. In general, invasive diagnostic procedures were required in 82.8% of cases; the diagnosis verification was achieved due to bronchoscopic sampling in 67.1% of cases, or surgical interventions – in 100% of cases.

Introduction. The natural course of some interstitial lung diseases (ILD) is characterized by progressive fibrosing phenotype resembling idiopathic pulmonary fibrosis (IPF). Until recently, the antifibrotic drug nintedanib was approved for treatment of the only fibrosing ILD which was IPF. A new indication for this drug which has been registered in Russian Federation in 2021 includes other fibrosing ILDs with progressive phenotype (PF-ILDs) and ILD associated with systemic scleroderma (SS-ILD).The aim of this publication is to describe general considerations of the decision of Multidisciplinary Expert Board on diagnosis and treatment of PF-ILDs including SS-ILD.Results. According to the extension in nintedanib use mentioned above, the Expert Board created an algorithm for diagnosis and treatment of patients with PF-ILDs and criteria for nuntedanib administration in PF-ILDs.Conclusion. Antifibrotic therapy is needed for patients with PF-ILDs with the failure of the stanrard therapy. In those patients antifibrotic treatment should be initiated as early as possible to better preserve the lung function.

Congenital lobar emphysema is a rare variant of lung malformation, which is characterized by hyperinflation of one or more lobes, usually diagnosed in early childhood. In this article, we demonstrate a clinical case of asymptomatic middle lobe emphysema diagnosed in a 16-year-old girl.

The absence of any specific symptoms, variety of radiologic and histologic changes in lungs and often poor physicians experience are the reasons for difficulties in diagnostics of idiopathic pulmonary fibrosis. The diagnostic criteria are constantly being discussed. The basis of this article is The White paper of Fleischner Society (published in “The Lancet Respiratory Medicine” 2017).

Introduction. Investigation of relationships between interstitial lung diseases (ILD) and development of heart failure would be useful for determining target therapy of heart failure (HF) among such patients. Aims.To study intracardiac hemodynamic and factors associated with its dysfunction among patients with ILD. 40 patients including: 34 with Chronic Hypersensitivity Pneumonitis (HP) and 6 with idiopathic pulmonary fibrosis (IPF). Methods. Pulmonary high-resolution computed tomography, echocardiography, spirometry, diffusing lung capacity for carbon monoxide (DLCO), bodypletysmography, blood oxygen (PaO ) were estimated. Results. 55.9% of patients with HP and all patients with IPF had a «cor pulmonale» with diastolic disfunction (DD), and 40% of them also had systolic dysfunction of right ventricle (RV). DD of left ventricle (LV) of I type was found among 10% of patients. 20% of patients were diagnosed with high pulmonary pressure (PP). The basal diameter of right ventricle (BDRV) correlated with DLCO (r=-0,718, p=0,004), PaO (r=-0,690, p=0,006), lung fibrosis (LF) (r=0,602, p=0,002). Tricuspid Annular Plane Systolic Excursion (TAPSE) correlated with mitral E/E’ ratio, tricuspid E/E’ ratio, total lung capacity (TLC) and LF (r=-0,753, p=0,002). Parameters of DD of RV significantly correlated with BDRV, TLC, DLCO, LF. Mitral E/E’ ratio correlated with DLCO and LF. PP corresponded with DLCO, DD of LV, LF (r=0,536, p=0,03) Conclusions. Correlation of LF with parameters of heart failure makes it one of the targets for ILD therapy as well as HF associated with ILD.

Interstitial changes in the lungs could be caused by vast majority of diseases including tuberculosis, sarcoidosis, hypersensitive pneumonitis, metastatic injury of the lungs, etc. Differential diagnosis of pulmonary dissemination remains an urgent and challenging clinical task. This article is a review of published literature and presentation of a clinical case of a patient with interstitial lung disease. The case demonstrates diagnostic difficulties in identification the cause of interstitial lung injuries. Interstitial lung injury was incidentally found in this patient and initially was considered as disseminated pulmonary tuberculosis. In-depth diagnostic work-up including lung tissue biopsy allowed diagnosis of lymphoid interstitial pneumonia associated with common variable immune deficiency. This case demonstrates common misdiagnosis of pulmonary tuberculosis in a patient with interstitial lung injury.

Aim: to perform a comparative analysis of the effi cacy of the inhaled and intravenous delivery of equivalent doses of bone marrow mesenchymal stem cells (BMMSCs) in rabbits according to the standard model of bleomycin pulmonary fi brosis.Materials and methods. After bronchoscopic instillation of bleomycin, 5 rabbits received intravenous transplantation of 2 × 106 allogeneic BMMSCs, other 5 rabbits – 2 × 107 MSCs inhaled via compressor nebulizer; control healthy and bleomycin group included 5 animals each.Results. Both groups treated with BMMSCs had a signifi cantly lower Ashcroft fi brosis index than the bleomycin control group. Expression of collagen in lung tissue in all groups with bleomycin injury was superior to healthy controls, but in animals underwent intravenous BMMSC transplantation collagen score was 0.74 points, and in inhaled treated group – 0.51 points, while in bleomycin controls – 2.1 point. Levels of TNF-α and TGF-β1 in BAL fl uids tended to decrease in treatment groups, but did not differ signifi cantly from control. A similar picture was observed in the cytological analysis of BAL.Conclusion. In general, both methods of delivering of BMMSCs to the lungs demonstrated similar therapeutic effects in inhibiting the development of experimental fi brosis, indicating that both intravenous and inhalational way of introduction can be used for subsequent clinical studies.

Combination therapy with long-acting β2-agonists (LABA) and long-acting muscarinic antagonists (LAMA) can reduce symptoms of chronic obstructive pulmonary disease (COPD) and the risk of future exacerbations. To date, the only fixed combination of long-acting bronchodilators, indacaterol/glycopyrronium, has demonstrated a significant reduction in dyspnea and in the risk of moderate and severe exacerbations of COPD in clinical trials when compared with the combination of salmeterol/fluticasone. Addition of inhaled steroids (ICS) to long-acting bronchodilators is recommended for patients with recurrent COPD exacerbations, especially in those with asthma-COPD overlap syndrome or history of elevated blood or sputum eosinophil levels. It is recommended to consider phenotype-specific therapy including roflumilast, N-acetylcysteine, and macrolides, in patients who continue to exacerbate despite being treated with LABA/LAMA or LABA/LAMA/ICS combinations. Withdrawal of inhaled corticosteroids is possible in patients with the low risk of exacerbation and in those with severe adverse events during ICS treatment. ICS should be withdrawn in a single step in patients with no repeated exacerbations during 12 months and with moderate bronchial obstruction (FEV1 ≥ 50% predicted). Stepwise withdrawal of ICS during 3 month with continuous dual bronchodilator therapy is recommended in COPD patients with severe bronchial obstruction (FEV1 ˂ 50% predicted) without frequent exacerbations in the previous year.

AIM:To evaluate the efficacy and safety of Spiriva (thiotropium bromide 18 microg for inhalation via a HandiHaler device) in patients with chronic obstructive pulmonary disease (COPD) of all severities in routine clinical practice in Russia.SUBJECTS AND METHODS:The study enrolled 407 patients (68 women and 339 men) with COPD who used thiotropium bromide (Spiriva) for 8 weeks. Most (72.3%) of the patients were aged 50-70 years; active smokers were 64.9%; ex-smokers were 27%; smoking duration averaged 38.6 pack-years; Severe, moderate, very severe, and mild COPD was observed in 38.6, 37.3, 18.4, and 5.7%, respectively. By the start of the trial, 305 (74.5%) had received concomitant therapy.RESULTS:After 8-week thiotropium bromide therapy, there was a significant increase in bronchial patency, as suggested by considerable increments in the postbronchial indices: forced expiratory volume in one second (FEV1) by an average of 290 ml (20.4%) of the baseline level during treatment and forced vital capacity (FVC) by 310 ml (12.1%). By the end of the trial, the mean increase in inspiratory capacity (IC) by 180 ml (8.07%) of the baseline value was indicative of decreased lung hyperinflation in the treated patients. The significant increment in mean FEV1, FVC, and IC was observed in patients with any severity of COPD.CONCLUSION:The RUSSE study has indicated that there may be very good results in patients with COPD of any severity and a steady-state positive effect just after 8-week thiotropium bromide treatment. This treatment improves bronchial patency and diminishes lung hyperinflation, thus improving the patients' health status and exercise endurance.

The authors describe the results of examination of 67 patients with exogenous allergic and idiopathic fibrosing alveolitis. Of these, 42 patients underwent plasmocytapheresis in addition to corticosteroid therapy. The clinical improvement, positive x-ray dynamics of pulmonary lesions, normalization of the altered laboratory indicators were noted in 39 out of the 42 patients treated with the use of plasmocytapheresis and in 15 out of 25 who received pharmacotherapy alone. The use of plasmocytapheresis at the inpatient stage of the treatment enabled the dose of corticosteroids to be reduced by 26 % of the initial on the average in 3/4 of the patients and subsequent hospitalization requirement to be noticeably decreased. Plasmocytapheresis did not produce any complications.