Comorbidities are important factors influencing the course and prognosis of interstitial lung diseases (ILDs). However, comparative data on the structure and clinical significance of comorbidities in idiopathic pulmonary fibrosis (IPF) and hypersensitivity pneumonitis (HP) remain limited.

The aim was to assess the prevalence and clinical significance of comorbidities in patients with IPF and HP.

Methods. This single-center prospective observational study included 621 patients (229 with IPF, 392 with HP) with a 12-month follow-up. Clinical, demographic, functional, and radiological characteristics, comorbidities, the Charlson Comorbidity Index (CCI), and the GAP (Gender, Age, Physiology) index were analyzed. All patients underwent pulmonary function testing, the 6-minute walk test (6MWT), echocardiogram, and high-resolution computed tomography scan of the chest.

Results. Most patients (66.2%) had 1 – 3 comorbidities, while 19.6% had ≥ 4 comorbidities. Patients with IPF had significantly higher CCI scores than those with HP (p < 0.001). The most common comorbidities were hypertension, cardiovascular disease (CVD), gastroesophageal reflux disease, pulmonary hypertension, and diabetes mellitus. Emphysema and obstructive airway diseases were more frequent in IPF (p < 0.05). A higher comorbidity burden and higher CCI scores correlated with higher GAP scores, shorter 6MWT distance, and more severe dyspnea (mMRC scale). The presence of ≥ 2 comorbidities was associated with increased mortality risk (AUC 0.587, p = 0.01), as was CCI ≥ 4 (AUC 0.608, p = 0.002; log-rank p = 0.007). CVD was associated with greater functional impairment and reduced exercise tolerance, while diabetes mellitus was an independent adverse prognostic factor, particularly in HP (OR 2.58, p = 0.016).

Conclusion. Comorbidities are highly prevalent in both IPF and HP and significantly influence disease course and prognosis. Assessment of comorbidity burden, including the CCI, may serve as an additional risk-stratification tool in patients with fibrosing ILDs.

Sarcoidosis is a systemic inflammatory disease of unknown etiology characterized by the formation of noncaseating granulomas, multisystem organ involvement, and T-cell activation at the site of granulomatous inflammation with the release of various chemokines and cytokines. The prevalence of sarcoidosis is 3 – 25 cases per 100,000 population. The mainstay of sarcoidosis therapy is corticosteroids, immunosuppressants, and drugs that affect oxidative stress and tumor necrosis factor. The aim of the article was to introduce the latest procedures for the diagnosis and treatment of sarcoidosis of various localizations, agreed upon in the Russian Federation. The target audience for these clinical guidelines are general practitioners, internists, pulmonologists, phthisiologists, pathologists, radiologists, and rehabilitation physicians. Methods. Each recommendation statement for diagnostic and therapeutic measures is assessed using evidence levels ranging from 1 to 5 and a recommendation strength rating scale for categories A, B, and C. The clinical guidelines also contain commentary and explanations for these recommendations, algorithms for the diagnosis and treatment of idiopathic pulmonary fibrosis, and reference materials. Conclusion. These clinical guidelines provide an overview of current knowledge on the etiology and pathogenesis, clinical manifestations, diagnosis, and treatment of sarcoidosis. These clinical guidelines were approved by the Scientific and Practical Council of the Ministry of Health of the Russian Federation in 2025.

The relevance of this work is determined by the need to implement the latest scientific advances in the problem of interstitial lung diseases (ILD) into medical practice. The aim of the work was to discuss the main updated guidelines for the classification of ILD by the European Respiratory Society (ERS)/American Thoracic Society (ATS). Results. The article presents the main clarifying changes to the international classification of ILD recommended by ERS/ATS experts. The new document proposes three main changes: expansion of the concept of “interstitial pneumonia” (IP) by including secondary cases; introduction of new subcategories of IP; identification of such subcategories as interstitial (fibrotic and non-fibrotic) disorders and disorders of alveolar accumulation. The article provides a detailed analysis of the essence of the proposed changes. Conclusion. A committee of multidisciplinary experts developed an updated structure for the classification of interstitial lung diseases. This structure expands the classification beyond idiopathic diseases, clarifies the terminology for bronchiolocentric interstitial pneumonia and alveolar-macrophage pneumonia, and further divides interstitial and alveolar filling disorders. Detailed information on various advances in molecular medicine is presented, and future research priorities are identified. These updates are expected not only to improve patient care but also to guide future research toward further understanding of these diseases, leading to further updates of this document by expanding the range of issues discussed and, ideally, providing a deeper biological basis for subgroups of the conditions.

The usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) is a key diagnostic hallmark of idiopathic pulmonary fibrosis (IPF). However, its prevalence and prognostic significance in other fibrosing interstitial lung diseases (ILDs), such as fibrotic hypersensitivity pneumonitis (fHP) and fibrotic ILD associated with systemic sclerosis (fILD-SSc), require a detailed comparative analysis.

The aim. To perform a comparative analysis of clinical and functional characteristics, as well as disease course, in patients with IPF, fHP, and fILD-SSc with the UIP pattern on HRCT.

Methods. This retrospective two-center study included a total of 554 patients: 219 with IPF, 280 with fHP, and 55 with fILD-SSc. HRCT patterns were classified as typical UIP and probable UIP by radiology experts who assessed the images. We compared clinical, functional, and hemodynamic parameters, and identified factors associated with the presence of the UIP pattern as well as predictors of disease progression over a 12-month follow-up period.

Results. The typical UIP pattern was identified in 43.4% of patients with IPF, 40.7% of patients with fHP, and 16.4% of patients with fILD-SSc (p < 0.001). Independent predictors of honeycombing in non-IPF fibrotic ILDs were male sex, high index scores GAP (Gender, Age, Physiology), digital clubbing, smoking pack-years, and the presence of cough (p < 0.05 for all). Male sex (OR – 2.25; p = 0.05) and GAP index scores (OR – 1.48; p = 0.012) remained the most significant factors in the multiple regression model. The UIP pattern was a predictor of disease progression in the fHP and fILD-SSc cohort, with the OR of 2.06 (95% CI – 1.07 – 3.96, p = 0.03).

Conclusion. The UIP pattern serves as a significant prognostic marker for progression in patients with fHP and fILD-SSc. This finding underscores the necessity for enhanced monitoring and consideration of early antifibrotic therapy in these patients.

Interstitial lung diseases (ILD) include idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF), both of which lead to severe respiratory failure within 3 – 5 years.

The aim of the study was to investigate the mRNA expression profile in blood mononuclear cells as a biomarker of IPF and PPF.

Methods. The 7-year prospective single-center study included patients (n = 110) with IPF (n = 16), PLF (n = 35), and nonprogressive ILD (n = 59). The pilot analysis of mRNA expression included patients (n = 24) with progressive PLF (n = 16) and IPF (n = 8) and a control group (n = 20). Since proinflammatory cytokines (IL-1A, IL-6, IL-8), interferon regulatory factors (IRF5, IRF7), KIF2C, CIITA, TYMS, HJURP, CDKN1A, UBE2C, BIRC5 proteins and pulmonary surfactants (SpA, SpB) are involved in immunity regulation, the expression level of 14 mRNA in blood mononuclear cells of patients with progressive pulmonary fibrosis was analyzed.

Results. Similar pathological patterns of mRNA expression of 9 genes (IL6, IRF5, IRF7, TYMS, HJURP, UBE2C, BIRC5, SpA, SpB) were revealed in patients with PLF and IPF compared to the controls. Expression of 5 genes (KIF2C, CIITA, CDKN1A, IL6, IL8) was significantly reduced. ROC analysis demonstrated that IL6 mRNA expression in peripheral blood mononuclear cells (AUC = 0.700) can serve as an informative noninvasive biomarker for the adverse course of ILD, including IPF and PPF.

Conclusion. A statistically significant decrease in KIF2C, CDKN1A, CIITA, IL6, and IL8 mRNA expression was detected in patients with progressive ILD. IL6 mRNA levels have acceptable prognostic significance (AUC = 0.700) and can be used as a noninvasive biomarker for progressive fibrosing lung diseases.

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease with a poor prognosis. The median survival after diagnosis does not exceed 2 – 3 years. One of the most significant complications of IPF is pulmonary hypertension (PH), the incidence of which is 20 – 84% and increases with the disease advancement.

The aim of the study was to comparatively analyze the clinical, functional and echocardiographic characteristics of patients with IPF depending on the degree of PH and to evaluate the diagnostic value of non-invasive parameters for detecting severe PH.

Methods. The observational study included patients (n = 121; median age – 69 years) with verified IPF. Depending on the pulmonary artery systolic pressure (PASP), the patients were divided into 3 groups. Group 1 (n = 35) included patients without PH (PASP < 35 mmHg). Group 2 (n = 59) included individuals with moderate PH (PASP 35 – 59 mmHg). Group 3 (n = 27) included patients with severe PH (PASP ≥ 60 mmHg). Clinical data, Charlson Comorbidity Index, 6-minute walk test (6-MWT), spirometry, diffusing capacity of the lungs for carbon monoxide (DLCO), high-resolution computed tomography (HRCT), and transthoracic echocardiography were assessed. Diagnostic accuracy was determined using ROC analysis, and the association with severe PH was determined using univariate and reduced multivariate logistic regression.

Results. As PASP increased, the incidence of cardiovascular events (26% vs 53% vs 74%, respectively; p < 0.001) and chronic heart failure (23% vs 47% vs 70%, respectively; p < 0.001) also grew, dyspnea progressed (modified Medical Research Council dyspnea scale – mMRC – 4 points: 8.6% vs 25% vs 41%, respectively; p = 0.049), desaturation rose during 6-MWT (7% vs 9% vs 12%, respectively; p = 0.002), and the right heart chambers dilated (the tricuspid annular systolic excursion (TAPSE) / PASP ratio was 0.697 vs 0.477 vs 0.317, respectively; p < 0.001). Spirometry parameters and DLCO did not differ between the groups. The degree of desaturation during the 6-MWT had the highest diagnostic value for detecting severe PH (AUC = 0.721: p = 0.005; sensitivity – 80%, specificity – 64% at the threshold > 9%). In the reduced multivariate model, desaturation remained the only independent predictor of severe PH (odds ratio – 1.237; 95% confidence interval – 1.086 – 1.437; p = 0.003).

Conclusion. Desaturation > 9% during the 6-MWT serves as a reliable non-invasive criterion for selecting patients with IPF for in-depth examination to detect severe

Tiotropium bromide is a long-acting antimuscarinic drug that is often referred to as an anticholinergic in clinical practice. Its high affinity for muscarinic receptors and slow dissociation provide a pronounced and prolonged bronchodilatory effect in patients with chronic obstructive pulmonary disease (COPD). Medications containing tiotropium bromide (international nonproprietary name) as the active ingredient have been successfully used in clinical practice for approximately 20 years, with their clinical efficacy and safety confirmed. A novel inhalation delivery of tiotropium bromide via metered-dose inhaler (MDI) has been developed.

Aim. This paper presents the results of an open-label, randomized, crossover comparative study of the pharmacokinetics, pharmacodynamics and safety of tiotropium bromide 9 μg/dose MDI (Bronpriva solopharm, Grotex LLC, Russia) and tiotropium bromide 18 μg dry-powder inhaler (Spiriva®, Boehringer Ingelheim International GmbH, Germany).

Methods. The study enrolled 70 eligible patients with a diagnosis of COPD with moderate bronchial obstruction (forced expiratory volume in 1 second (FEV₁) in the post-bronchodilator test: 50% ≲ FEV₁ < 80%; FEV₁ / forced vital capacity (FVC) < 70%). Patients were randomized into 2 equal groups and received therapy with the tiotropium bromide 9 μg/dose and the tiotropium bromide 18 μg dry-powder inhaler. Pharmacodynamic equivalence was measured based on statistical analysis (ANOVA) of pulmonary distribution parameters (FEV₁, peak expiratory flow) of tiotropium bromide.

Conclusion. Tiotropium bromide 9 μg/dose MDI and tiotropium bromide 18 μg dry-powder inhaler were found to be pharmacodynamically equivalent. Tiotropium bromide delivered via MDI has a favorable safety profile.

In real-world clinical practice, mild asthma is the most common type of asthma but selection of the basic therapy for these patients can present certain challenges. Monotherapy with inhaled corticosteroids (ICS) in the form of finely dispersed metered-dose aerosols (e.g., ciclesonide) may prove highly effective in adolescents with poorly controlled mild asthma.

The aim of this multicenter, open-label study was to evaluate the efficacy of ciclesonide in different dosing regimens for achieving and maintaining asthma control in adolescents with initially uncontrolled asthma.

Methods. An open, prospective, non-comparative study lasting 90 ± 7 days included patients (n = 129: 69.8% boys; mean age 14.2 ± 1.7 years) with uncontrolled mild atopic asthma (household, epidermal sensitization, and tree pollen in some patients) in 14 centers. The patients received background therapy with ciclesonide 160 μg per day. Asthma control tests (Global Initiative for Asthma (GINA) questionnaires and the Asthma Control Test (ACT) were completed at 3 visits. The number of asthma exacerbations, days with use of emergency medications and/or school absences due to asthma symptoms, and adverse events were taken into account. Spirometry with a salbutamol challenge was performed, and absolute eosinophil blood counts were measured at Visits 1 and 3. Subanalyses were performed for the subgroups of patients who were switched to ciclesonide 80 μg/day at Visit 2 (n = 26) by the treating physician and for those with tree pollen allergy (n = 49).

Results. The proportion of individuals with controlled asthma increased with regular ciclesonide treatment: 94 (77.7%) at Visit 2 and 111 (95.7%) at Visit 3. No significant differences in asthma control criteria were found between the groups with and without tree pollen allergy. When comparing spirometric indices at Visits 1 and 3, the proportion of patients whose baseline forced expiratory volume in 1 second was > 80% of predicted value increased to 100% (p = 0.026 compared to Visit 1 – 34.6%), and the proportion of individuals with a negative result in the salbutamol test increased to 83.6% (p = 0.0001 compared to Visit 1 – 12.4%). No changes in blood eosinophil content were shown between Visits 1 and 3 (Me (Q25; Q75)): 310 (180; 500) and 290 (150; 450) cells/μl. The results of questionnaires, spirometry and the number of emergency inhalations showed maintained asthma control in the subgroup of patients who received ciclesonide at a dose of 80 μg per day after Visit 2. No adverse events of clinical significance or requiring discontinuation of ciclesonide were reported.

Conclusion. Background therapy with ciclesonide (Asmalib® Air, metered-dose aerosol for inhalation, LLC PSK Pharma, Russia) in adolescents has a positive effect on asthma control (assessed using the ACT and GINA) and spirometry results. A reduction in the frequency of rescue medication inhalations was also noted.

Sarcoidosis is a chronic inflammatory disease characterized by the formation of granulomas in various organs and body systems. It manifests with a variety of symptoms, involving lungs, joints, skin, eyes, and other organs.

The aim of this review was to discuss a new approach to long-term sarcoidosis therapy.

Results. Treatment of sarcoidosis includes various approaches aimed at suppressing inflammation and granulomatosis progression, and preventing complications such as organ and system failure and fibrosis, but each approach is advisory in nature. For over half a century, corticosteroids have remained the standard first-line treatment, but their long-term use is associated with serious side effects. Methotrexate and other immunosuppressants offer alternatives to corticosteroids and are used when the latter are ineffective or intolerable. However, the choice of drug is personalized. Long-term glucocorticoid use is associated with an increased risk of developing diabetes, hypertension, osteoporosis, and infections. Therefore, it is important to monitor side effects and promptly adjust treatment strategies.

Conclusion. The current position suggests switching to methotrexate after initial hormonal therapy, which provides long-term disease control with fewer side effects.

Lymphangioleiomyomatosis (LAM) is a rare, progressive, neoplastic lung disease characterized by excessive smooth muscle cell proliferation, leading to the development of cystic lung lesions. The disease is caused by mutations in the tuberous sclerosis complex (TSC1) or TSC2 genes. It predominantly affects women. LAM is diagnosed in patients with tuberous sclerosis (TS-LAM) and as a sporadic disease (sporadic LAM, sLAM).

The aim of this study was to review the literature and present clinical data on patients with LAM.

Methods. A brief literature review was prepared using Scopus and PubMed databases. Data from our own clinical observations are presented.

Results. A literature review and two clinical observations of patients with LAM are presented, stratifying factors of an unfavorable prognosis. The first patient was premenopausal and had multiple thin-walled air cysts, complicated by recurrent pneumothorax and left kidney damage (angiomyolipoma). The second patient was taking combined oral contraceptives and presented with progressive respiratory failure with diffusely distributed progressive thin-walled cysts in the lungs, accompanied by a decrease in the lung diffusion capacity.

Conclusion. Initial diagnosis of LAM requires a multidisciplinary approach, and once detected, a thorough examination and close monitoring are necessary.

Lymphangioleiomyomatosis (ЛАМ) is a rare, systemic and progressive tumor disease characterized by abnormal proliferation of fusiform smooth muscle cells. These cells penetrate into the lungs, kidneys, and lymphatic system, which leads to the development of diffuse thin-walled lung cysts, renal angiomyelitis, and various lymphatic abnormalities. There are 2 main variants of ЛАМ: sporadic ЛАМ (sЛАМ) and ЛАМ associated with tuberous sclerosis (TS). The disease has a pronounced gender predisposition and affects mainly women of reproductive age. ЛАМ in men is extremely rare.

The aim of this study was to demonstrate clinical case of long-term follow-up of a patient with ЛАМ associated with TS with the background of everolimus therapy.

Results. The presented clinical observation demonstrates a relatively benign course of the disease in a patient with TS diagnosed in childhood, with skin lesions, an increase in azotemia against the background of rupture of renal angiomyolipoma, and ЛАМ detected in adulthood. Timely administration of mTOR inhibitors was associated with recovering renal function and no significant increase in pulmonary function impairment.

Conclusion. A multidisciplinary approach to the examination and treatment of patients with TS is a key factor in timely diagnosis verification, timely therapeutic tactics, and reducing the risk of progressing organ dysfunction.

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease of unknown etiology, most commonly occurring in the elderly. The main factors leading to the development of IPF are environmental factors, viruses, and genetic predisposition. The «gold standard» for diagnosis is high-resolution computed tomography. Antifibrotic drugs such as nintedanib and pirfenidone are used as pathogenetic therapy.

The aim was to present a patient with a long history of dyspnea and heart failure, in whom the diagnosis of IPF was established only upon hospitalization due to worsening dyspnea. High-resolution computed tomography revealed a characteristic pattern of “honeycomb lung”.

Conclusion. The article demonstrates the difficulties in diagnosing IPF in old age associated with polymorbidity (chronic obstructive pulmonary disease, chronic heart failure), which leads to a late start of specific therapy. 

Over the past decade, the rapid growth in popularity of electronic nicotine delivery systems has led to an increase in lung injury cases and a new condition known as e-cigarette or vaping use-associated lung injury (EVALI).

The aim of this study was to present the first case of e-cigaretteassociated lung injury reported in a hospital setting.

Results. A young patient was admitted to the hospital on an emergency basis with severe respiratory complaints. Initial radiographic findings were normal. Over the course of one year, the patient developed progressive respiratory failure, leading to widespread pneumofibrosis, which ultimately resulted in death.

Conclusion. Based on the identified combination of emphysema, pulmonary fibrosis, and histologically confirmed alveolitis, along with a history of e-cigarette smoking, a diagnosis of toxic acute lung injury (EVALI syndrome) with a histological pattern of diffuse alveolar damage, resulting in pulmonary fibrosis, was verified.

Expanding the range of biological drugs for the treatment of patients with severe asthma requires rationalizing a personalized approach to treatment to account for the potential risks of adverse events. Information on the development of drug-induced sarcoid-like syndrome during anti-IL-4, -13 therapy is limited to isolated publications and requires a detailed analysis of each specific clinical case. This is necessary to further develop a patient profile associated with higher risks of adverse events related to anti-IL-4, -13 therapy, as well as to develop a rational algorithm for dynamic laboratory and instrumental monitoring and to define its timing.

The aim was to investigate the emergence of drug-induced sarcoid-like syndrome associated with anti-IL-4, -13 treatment in a patient with severe asthma and chronic rhinosinusitis with nasal polyps (CRSwNP), as well as to evaluate the safety and efficacy profile of alternative biological therapies.

Conclusion. The therapeutic potential of anti-IL-4, -13 therapy in patients with bronchial asthma and CRSwNP is undisputed. Given the risk of adverse events associated with biological therapy, strict frequent monitoring of laboratory and instrumental data is required. Further investigation of the efficacy and safety of biological therapy in asthma and CRSwNP will help determine the optimal timing of treatment response and monitoring intervals, identify possible predictors of adverse events, and develop personalized therapeutic approaches.

Sarcoidosis is a systemic inflammatory disease of unknown origin that can progress to fibrocavernous disease. Published data on antifibrotic therapy for fibrotic sarcoidosis are contradictory, largely due to an insufficient number of studies. In randomized trials, nintedanib has demonstrated efficacy in reducing the progression of interstitial lung disease; however, it has not shown a positive effect specifically in fibrotic sarcoidosis. Some individual studies have reported beneficial effects of antifibrotic therapy in cases of fibrotic sarcoidosis. Due to the limited and conflicting data, each observation of antifibrotic therapy in fibrotic sarcoidosis is valuable.

The aim. Here, we describe our experience of using antifibrotic therapy in a patient with fibrotic pulmonary sarcoidosis.

Methods. The patient, a 66-year-old woman with sarcoidosis-associated pulmonary fibrosis, has been receiving antifibrotic therapy (nintedanib 150 mg twice daily) since October 2022. Efficacy and safety were assessed before initiation and after 12, 24 and 32 months of antifibrotic therapy.

Results. The respiratory deterioration slowed down, evidenced by an increased distance in the 6-minute walk test. Pulmonary function decline was also reduced, with no decrease in FEV₁, FVC, FRC, or RV. Computed tomography scans revealed no progression in the volume of pulmonary fibrosis.

Conclusion. The use of antifibrotic therapy was associated with stabilization of pulmonary function and no progression of pulmonary fibrosis. Further studies on antifibrotic therapy in fibrotic sarcoidosis are warranted.

The expansion of available treatment methods for community-acquired pneumonia (CAP) is more relevant than ever. Drugs with pronounced anti-inflammatory, antioxidant, and regenerating properties are needed to help prevent lung damage and minimize potential complications as part of complex therapy. The purpose of the publication of the resolution of the Board of Experts was to discuss the efficacy and safety of hexapeptide succinate (Amberwin® Pulmo) in the treatment of respiratory diseases, primarily CAP. The Board of Experts met on February 26, 2026 in Moscow under the chairmanship of the Doctor of Medicine, Professor, Academician of Russian Academy of Sciences S.N.Avdeev. Results. Phase III clinical trials demonstrated the following advantages of hexapeptide succinate: anti-inflammatory, immunoregulatory, antioxidant, reparative and organoprotective effects; rapid reduction in the level of pro-inflammatory cytokines, acceleration of clinical effect, reduction of hospitalization time, and cost-effectiveness. Conclusion. The data presented justify the inclusion of hexapeptide succinate in clinical guidelines for the treatment of CAP in adults.