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Antineutrophil cytoplasmic antibodies associated vasculitis (ANCA-AV) is a systemic necrotizing granulomatous vasculitis affecting mainly small-caliber vessels. ANCA-AV occupy a special place among systemic vasculitis, which is characterized by a highly active life-threatening course of the disease, requiring rapid differential diagnosis and aggressive immunosuppressive therapy. The ANCA-AV group consists of 3 nosologies: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. The “calling card” of ANCA-AV is the lesion of the upper respiratory tract, especially the ENT organs (70–100 % of patients). The nasal cavity and paranasal sinuses are the most common areas of lesion in the head and neck (85–100 %), whereas ear damage occurs in about 35% (range, 19–61 %) of cases. Lesion of the ENT organs is typical for the debut of ANCA-AV, which makes early diagnosis difficult, since diseases of the upper respiratory tract are extremely common in all age groups. Diagnosis verification occurs mainly at the stage of generalized involvement of many organs and systems, causing severe course with the development of pulmonary-cardiac and renal insufficiency, which lead to the death of the patient. The main ENT manifestations of ANCA-AV can be grouped into several groups: sinonasal, otological, tracheobronchial, oral cavity lesions and others. Pseudotumors are often found in ANCA-AV. They are characterized by the appearance of parapharyngeal, parotid, submandibular, paratracheal volumetric formations. As a rule, the appearance of tumor-like formations is observed at an early stage of the disease and is associated with the presence of antibodies to proteinase 3, systemic manifestations of vasculitis. Pseudotumor in the ENT region may be accompanied by secondary neuropathies of cranial nerves, destruction of bone tissue, which requires histological verification of the disease.

The aim – to study risk factors for severe COVID-19 in patients with rheumatic diseases (RD).Patients and methods. The study included medical histories of 464 patients with RD who were admitted at the V.A. Nasonova Research Institute of Rheumatology from September 27, 2021 to April 26, 2023Results. Age over 60 years, hypertension, obesity, lung disease, chronic kidney disease, coronary heart disease, diabetes mellitus, acute cerebrovascular accident or a history of pulmonary tuberculosis increase the risk of hospitalization in patients with RD with COVID-19 by 3–5 times. In addition, with an increase in the number of concomitant diseases, an increase in the risk of hospitalization was noted by 2–6 times. Taking glucocorticoids, including at a dose of ≥10 mg per day for prednisolone, mycophenolate mofetil and rituximab, leads to an increase risk of hospitalization by 1.5–4.5 times, while patients taking hydroxychloroquine or tumor necrosis factor α inhibitors was more often required outpatient treatment.Conclusions. It has been established that older age, the presence of comorbid pathology and the use of glucocorticoids, including at a dose of ≥10 mg per day for prednisolone, mycophenolate mofetil and rituximab, are risk factors for severe COVID-19.

The article presents a review of current data on arteriovenous thrombosis risk factors in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Thrombotic complications are among the most frequent prognostically unfavorable factors in AAV. In general, patients with AAV are at greater risk of mortality from cardiovascular events compared with the general population and patients with other forms of systemic vasculitis. Understanding the mechanisms of thrombosis, as well as pathogenic factors that cause an increased risk of arterial and venous thrombosis in AAV, can improve the quality of management of these patients. Therefore, the question of the need for primary and secondary prevention of thrombosis in AAV is of great practical importance. The presented data show the need to estimate the probability of thrombosis in patients with AAV, taking into account risk factors, as well as the influence of the disease itself and ongoing therapy. Assessment of the risk of thrombosis (both venous and arterial) is necessary for the timely administration of adequate preventive treatment of thrombotic complications in AAV.

Depending on the epitope specificity of antineutrophil cytoplasmic antibodies (ANCA) and the presence or absence of a granulomatous inflammation. Patients with ANCA-associated vasculitis (AAV) show variability in clinical manifestations and prognosis depending on the epitope specificity of ANCA and the presence or absence of granulomatous inflammation. In this regard, it is important to identify the clinical and immunological phenotypes of AAV and a personalized approach to treatment. Microscopic polyangiitis (MPA) with antibodies to myeloperoxidase (aMPO) has a relatively high incidence of lung involvement and pulmonary fibrosis. We present our own clinical case of pulmonary fibrosis in MPA with aMPO and rheumatoid factor. Literature data are discussed. In cases of early arthritis and interstitial pneumonia, the possibility of AAV should be considered, a detailed examination is necessary to identify pathognomonic signs of AAV, including asymptomatic ones. Before prescribing immunosuppressants, the epitope specificity of ANCA should be determined. Treatment with rituximab, mycophenolate mofetil and nintedanib is discussed.

Patients with ANCA-associated vasculitis (AAV) cause extreme alertness during the coronavirus disease 2019 (COVID-19) pandemic, associated with many factors: the initial damage to the respiratory system (upper respiratory tract, lungs) and kidneys, immunosuppressive treatments, difficult prognosis of COVID-19 with the risk of AAV exacerbation. We present a clinical case of а moderate COVID-19 in a patient with granulomatosis with polyangiitis, who received anti-B cell therapy with rituximab (RTX) for a long time. Coronavirus pneumonia developed one year after RTX, while B-lymphocyte depletion persisted. In order to achieve an adequate antiviral immune response and prevent hyperinflammation, treatment was carried out with antiviral drugs, anticoagulants, convalescent plasma, human normal immunoglobulin, and interleukin-6 antagonist tocilizumab. Possible predictors of severe COVID-19 in patients with AAV are discussed.

The World Health Organization announced a new coronavirus pandemic on 11 March 2020. It is known that the most frequent clinical manifestation of a new coronavirus infection is lung damage with the evolution of pneumonia, as well as respiratory distress syndrome. The highest risk of severe traction and death is known to occur in the immunosuppressive group of patients. These patients include rheumatologically patients. Currently, there is no complete understanding of the pathogenesis of the development of covid-19, as well as the pathogenesis of the development of autoimmune diseases. In a pandemic, the question of how the coronavirus infection proceeds in patients on immunosuppressive therapy, in particular on anti-B cell, is relevant. In our case report presents a 69-year-old female with ANCA-associated vasculitis remission having received a B-cell-depleting therapy with rituximab (RTX), who had an ocular lesion as a clinical manifestation of Coronavirus disease 2019 (COVID-19). Two months after RTX treatment, COVID-19 was diagnosed. The patient had extrapulmonary manifestations including central nervous system involvement and ocular symptoms such as conjunctivitis, without respiratory pathology. In this clinical observation, the patient’s disease manifested itself with right-sided conjunctivitis, which is a rare manifestation as the first symptom. This article describes the features of the course and outcome of the disease, as well as, the protective effect of RTX in course of COVID-19 is discussed. A comparative analysis of cases of COVID-19 with eye damage is being carried out. This clinical observation is of great value for doctors of all specialties, especially ophthalmologists. A multidisciplinary approach is crucial to manage COVID-19.

This article describes the various forms of inflammatory lesions of the aorta and large arteries, including chronic periaortitis, as well as the diagnostic methods are considered. Large vessel vasculitis represent the most common entities, however, there is also an association with other rheumatological or inflammatory diseases, drug-induced or paraneoplastic entities. Instrumental imaging modalities play an important role in the diagnosis.

Actual problems of rheumatology include the possibility of developing a wide range of long-term consequences of COVID-19, so-called post-COVID syndrome (PCS). The results of our own research are presented, during which among 45 patients over 18 years with rheumatic diseases (RD) PCS was detected in 20% cases: 6 of them had ANCAassociated vasculitis (AAV) and one each for rheumatoid arthritis, juvenile arthritis and osteoarthritis. In AAV cases PCS was more severe and varied than in other RD: lung lesions according to computed tomography (up to the damage 75% of the lung parenchyma), skin an d nervous (Guillain–Barré syndrome) lesions, myalgias, arthralgias were present. In most patients PCS ended in recovery, sudden death occurred only in the patient with AAV. The frequency of RD’s relapses in patients with PCS was 9%. Relapses were not noted in AAV, against the background of anti-B cell therapy with rituximab. Our own results and literature data indicate that patients with RD after COVID-19 need careful continuous monitoring to detect long-term complications of coronavirus infection and early diagnosis of relapses of the underlying disease.

The present report illustrates efficacy of rituximab (RTX) in granulomatosis with polyangiitis (GPA) with severe lung involvement. Female patient, 45 years old, was ill since March 2016, her disease manifested at the onset with fever, recurrent epistaxis, otitis media, mastoiditis, conjunctivitis and arthritis. Thoracic CT scan showed multiple decaying pulmonary infiltrates. The presence of PR3-ANCA confirmed the diagnosis of GPA. Induction therapy included high doses of glucocorticoids, cyclophosphamide (total dose 4 g), with following azathioprine and mycophenolate mofetil. Lung disease continued to progress with emerging extensive infiltrates and forming a giant cavity with air-fluid level in the right lung. Further treatment included antibiotics followed by surgical draining of lung cavity in December 2018. Fever, necrotic rhinitis and otitis persisted despite treatment, lab findings included red blood cells in the urine, C-reactive protein 90 g/l, thrombocytosis 740×109 /l, anemia (Hb 80 g/l). RTX 2 g and intravenous immunoglobulin were initiated in December 2018, a second course of RTX (0.5 g) was administered 4 months later. Patient’s condition was gradually improving, CT scan at 6 months after RTX treatment showed fibrous tissue in the area of former cavity. One year later, total RTX dose was 3.5 g, further regression of changes and GPA remission were achieved. There were no adverse reactions. Anti-B cell therapy with RTX is a safe and highly effective option in GPA patients with severe destructive lung disease, potentially curative even in cases of giant pulmonary cavities.

Objective. Currently, the issues of the effect of anti-B cell therapy or inhibitor of interleukin 5 on the risk of COVID-19 infecting and outcomes in patients with ANCA-associated vasculitis (AAV) has not been completely studied. We present an analysis of the COVID-19 course and outcomes in AAV patients treated with rituximab or mepolizumab from one rheumatology center registry.Methods. From November 11 to November 15, 2020, a cross-sectional study was conducted using telephone and online surveys, and information was collected from all 128 AAV patients treated with rituximab in V.A. Nasonova Research Institute of Rheumatology. Patients mean age was 51 (20–81) years, 61.7% were women. Granulomatosis with polyangiitis (GPA) was diagnosed in 58 patients, microscopic polyangiitis (MPA) – in 38, eosinophilic granulomatosis with polyangiitis (EGPA) – in 24 (including 54.2% of ANCA-negative cases), and AAV with uncertain nosological affiliation – in 8 patients. Due to the disease activity or a high risk of AAV recurrence during the pandemic rituximab was prescribed in 60/126 (47.6%) patients, and mepolizumab – in 6 cases.Results. In the spring of the pandemic (until May 2020), the incidence of COVID-19 in AAV patients treated with rituximab was 4.3%, the disease course was relatively favorable. All patients recovered. At month 3–6, antibodies to SARS-CoV-2 IgG persisted in only 1 out of 4 patients. Since September 2020, the incidence has increased 3-fold, with a more severe course of COVID-19. In total, in the period until November 11, 2020, COVID-19 was diagnosed in 17.2% (22/128); the mean age of patients was 55 (25–81) years; 54.5% were women. 21/22 patients were on rituximab therapy, 2 patients had mepolizumab therapy (including 1 case after previous rituximab therapy). COVID-19 incidence was lower in patients with GPA (15.5%) vs MPA and EGPA (21.1% and 20.8% respectively). The mortality rate was 13.6%, including 2 patients with MPA and 1 patient with GPA. When analyzing the 5-year survival rate according to the registry of AAV patients treated with rituximab, prognosis worsening was noted; in 2020 there were 3 deaths due to COVID-19, in the previous 5 years – only 2 deaths.Discussion. Taking into account the fact the mechanisms of AAV and severe COVID-19 are largely synergistic (primarily in the context of microvascular COVID-19 lung vessels obstructive thromboinflammatory syndrome as manifestation of the acute inflammatory syndrome), the activity of AAV can potentially contribute to the disease onset and a severe course of COVID-19. Given the previously published information on the use of rituximab during the COVID-19 pandemic for various diseases, it seems that B cell depletion, without reducing the risk of infection, may have a protective effect with regard to the risk of severe/catastrophic COVID-19, which, however, can be insufficient in AAV patients. Further analysis of COVID-19 cases in patients with AAV and other immuno-inflammatory rheumatic diseases is exceptionally important.

Introduction. In children with rheumatic diseases, severe fungal infections (invasive mycoses – IM) are not well understood.Objectives. To analyze risk factors, disease course of IM in children with systemic rheumatic diseases.Materials and methods. For diagnosis of IM were used criteria EORTC/MSGERC, 2019. We reviewed the literature over the past 15 years on IM in children with rheumatic diseases from the international databases Pubmed and Web of Science.Results. In retrospective multicenter study were included 8 children with IM and systemic rheumatic diseases: ANCA-associated vasculitis (n=4), systemic lupus erythematosus (n=3), juvenile rheumatoid arthritis (n=1). Median age was 13,5 (8-17) y., boys – 67%. Invasive aspergillosis was diagnosed in 5 patients and invasive candidiasis – 3. The risk factors of invasive mycoses were high rheumatic disease activity (100%), corticosteroids (prednisolone ≥ 0,3 mg/kg/d) use for ≥21 d (87,5%), immunosuppressive therapy (87,5%), recent (≤ 2 weeks) pulse steroid therapy (75%), hemophagocytic lymphohistiocytosis (62,5%), prolonged (≥ 10 days) severe neutropenia (≤ 0,5х109/l) (62,5%), and prolonged (≥10 days lymphopenia (≤ 1,0х109/l) (37,5%). In patients with invasive aspergillosis the involved organ was the lung, in patients with invasive candidiasis a candidemia was diagnoses. All patients received antifungal therapy. The overall 30 days survival rate was 37,5%.Сonclusions. Children with high rheumatic diseases activity and intensive treatment with immunosuppressive agents should be considered as patients with a high risk of invasive mycoses with a high mortality.

Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.

The aim of the study – to determine the diagnostic value of serum levels of endothelin-1, alpha-actin-2 and elastin in patients with primary systemic vasculitis. Materials and methods. Twenty eight patients with ANCA-associated vasculitis, 8 patients with polyarteritis nodosa of moderate or high vasculitis activity (BVAS >11) and 26 healthy subjects (control group) were examined. Serum levels of endothelin-1, alpha-actin-2 and elastin were determined by immunoassay using commercial kits of Biomedica, Biorbyt and Bender Medsystems. Results. The level of endothelin-1 in patients with primary systemic vasculitis did not significantly differ from that of the control group, but its level (0.40 ± 0.33 pmol/l) was significantly higher in patients with kidney involvement (n = 15) compared with patients without kidney damage (0.28 ± 0.22 pmol/l, P = 0.04) and the control group (0.27 ± 0.10 pmol/l, P < 0.01). The level of alpha-actin-2 in the group of patients with ANCA-associated vasculitis was twice as high as in the control group (P < 0.05), which can be used for diagnosis this group of diseases (AUC 0.96 ± 0.16; 95 % CI 0.60–1.00; sensitivity 83 %, specificity 84 %, P

The study objective is to demonstrate the difficulty of differential diagnosis in pulmonary-renal syndrome using a clinical case as an example.Materials and methods. Male patient A., 68 years old, retired, was hospitalized at the N.I. Pirogov City Clinical Hospital № 1 in December of 2017 with complaints of inefficient cough, fever of 39 °С, weakness, apnea, weight loss up 10 kg in 3 months. Examination revealed skin and mucosa paleness, calf edema, heart beat of 102 bpm, normal rhythm, arterial pressure 130/80 mm Hg, respiratory rate 22 breaths per min. Auscultation revealed harsh respiration in the lungs, weakened in the lower parts, fine moist rales. Anemia (hemoglobin – 53 g/l, erythrocytes – 1.85 × 1012/l, serum iron – 3.1 µmol/l), elevated urea up to 41.4 mmol/l, creatinine up to 843.1 µmol/l (glomerular filtration rate – 6 ml/min/1.73 m2), leukocytes up to 12.5 × 109/l, С-reactive protein up to 124.96 mg/l were diagnosed. Clinical urine analysis showed proteinuria 0.47 g/l. Computed tomography of the chest revealed pronounced infiltrative changes in tissues of both lungs, more on the right, alveolitis, bronchiolitis in the middle lobe on the right, 5th segment on the left. Lymphadenopathy mediastinal was diagnosed. After examination (multiple bacteriological blood, sputum tests, interferon-gamma release assay, echocardiography, bronchoalveolar lavage, sterna puncture, esophagogastroduodenoscopy, colonoscopy, etc.), oncological pathology, tuberculosis of the lungs, sepsis, infections endocarditis and other infectious pathologies were excluded. Antibacterial courses prescribed earlier were ineffective. Immunological blood test revealed high titers (1:1280) of antineutrophil cytoplasmic antibodies (ANCA) with perinuclear fluorescence type (myeloperoxidase specificity), negative antibodies to glomerular basal membrane which allowed to diagnose ANCA-associated vasculitis.Results. Considering the data of clinical, lab, and instrumental examination, the patient was diagnosed with microscopic polyangiitis, ANCA-associated, affecting the lungs (disseminated interstitial lung disease with bronchiolitis) and kidneys (rapidly progressive glomerulonephritis), intrathoracic lymphadenopathy, activity grade III (BVAS index – 23 points). Grade II respiratory failure. Chronic kidney disease 5D (glomerular filtration rate – 6 ml/min/1.73 m2). Grade II arterial hypertension, risk 4. Grade II pulmonary hypertension. Chronic heart failure 2А, functional class IV. Mixed anemia (iron-deficient, chronic disease), severe. Disseminated polyposis of the colon (hyperplastic type). At the hospital, antibacterial drugs (cefoperazone sulbactam), antifungal (fluticasone) were administered, anemia was corrected (iron-containing drugs and erythropoietin, hemotransfusion), hemodialysis. Cyclophosphane 400 mg was administered intravenously, a week later – 800 mg. Methylprednisolone (60 mg/day), co-trimoxazole (480 mg 3 times a week) were prescribed. A pronounced improvement was observed due to the therapy: body temperature normalization, decreased apnea, cough, weakness, increased appetite. The patient was discharged with recommendation for continuation of cytostatic therapy per the regimen and prescription for programmed hemodialysis at the place of residence.Conclusion. This clinical case demonstrates a necessity of considering ANCA-associated vasculitis during differential diagnosis of pulmonary-renal syndrome. Timely diagnosis and active cytostatic therapy play a principal role in treatment and promote deceleration of disease progression and improve prognosis.

Objective. To compare the frequency, clinical features and outcomes of renal involvement in ANCA-associated vasculitides (AAV) in patients with antibodies against proteinase-3 (pr3-ANCA) and myeloperoxidase (MPO-ANCA). Materials and methods. In our retrospective study we enrolled 264 patients, 94 males and 170 females, median age 53 [36; 62] years. Among them 157 were pr3-ANCA positive and 107 were MPO-ANCA positive. AAV was diagnosed according to ACR criteria and Chapel Hill consensus conference definition (2012). Median follow up was 44 [18; 93] months. We assessed baseline BVAS and VDI by the end of the follow up. Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and daily proteinuria were estimated. Diagnosis and stage of chronic kidney disease (CKD) and acute kidney injury (AKI) were established according to KDIGO guidelines (2012) and Scientific Society of Russian Nephrologists (2016). Results. Renal involvement was present in 181 (68.6%) patients, and its frequency was similar in pr3-ANCA and MPO-ANCA subgroups. Patients with MPO-ANCA developed rapidly progressive glomerulonephritis and hypertension significantly more often than patients with pr3-ANCA: 50.7% vs 35.6% (p=0.049) and 46.1% vs 29.8% (p=0.029) respectively. At disease onset, median sCr was significantly higher and eGFR was significantly lower in patients with MPO-ANCA (p

Aim. To evaluate clinical features and outcomes of renal involvement in patients with microscopic polyangiitis (MPA). Materials and methods: We enrolled 99 patients with MPA, diagnosed in accordance with the algorithm of the European Medicines Evaluation Agency (EMEA) and the Chapel Hill consensus conference definition (2012). Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and proteinuria were estimated. Frequency of rapidly progressive renal failure (a twofold increase in the sCr level in ≤3 months) was regarded as the clinical equivalent of rapidly progressive glomerulonephritis (RPGN). Results and discussion. Renal involvement was present in 92 (92.9%) patients. RPGN developed in 51 (55,4%) patients. The most common features of kidney involvement were hematuria and subnephrotic proteinuria. Arterial hypertension was revealed in 32 (34.7%) patients and was associated with RPGN (p

Systemic vasculitis is a heterogeneous group of inflammatory diseases, which are classified according to the diameter of the affected vessels. The treatment of systemic vasculitis is no longer empirical, because of increasing number of randomized clinical trials in this field. In recent years, there was a trend to limit the cumulative dose of glucocorticosteroids and immunosuppressive drugs, partially through biological drugs usage. However, biologic therapy is not always superior to combination of glucocorticosteroids and cytotoxic drugs. The efficacy of different biologic drugs varies in patients with different forms of systemic vasculitis, which creates the ground forpersonalized therapy of these diseases. Another serious problem is the absence of strict criteria to immunosuppressive therapy intensification, especially in vasculitis, affecting large vessels, due to the lack of reliable laboratory markers of disease activity. The article reviews modern approaches to the treatment of some types of systemic vasculitis, including ANCA-associated vasculitides, giant cell arteritis, Takayasu`s arteritis and cryoglobulinemic vasculitis.

Nowadays, there is no doubt about the relationship between immune inflammation and the development of thrombosis due to the similarity of many pathogenetic machanisms underlying both pathological processes. Systemic vasculitis, along with other immuno- mediated inflammatory diseases, represent one of the most clear examples of such interaction. Thrombotic complications remain one of the most serious and life-threatening conditions that occur in patients with autoimmune diseases, in particular systemic vasculitis. This serves as powerful stimulus for studing the problem of hypercoagulation, which often accompanies the course of systemic vasculitis. Of interest are thrombosis that occure both in the injury of large vessel and in the involvement of medium and small vessels in the pathological process. According to the literature, thrombotic and thromboembolic complications are most common in ANCA-associated vasculitis and Behçet’s disease. This review discusses current studies regarding features of clinical picture and mechanisms of thrombosis development in systemic vasculitis. Because of these studies it became clear that for some vasculitis a high frequency of both arterial and venous thrombosis is characteristic. At the same time, other vasculitis are accompanied by high risk of only venous thrombosis. Finally, thrombosis and thromboembolism are quite rare complications for some vasculitis. Among the presented mechanisms of thrombosis involvement, disruptions of the hemostasis are widely considered. For examples, the presents the results of researches, which is actively discussed the role of neutrophil extracellular traps and antiendothelial cell antibodies in the development on thrombotic complictions. Special attention is paid to the possible role of some disorders of hemostasis, such as polymorphism V coagulation factor G1691FA and prothrombin G20210A. To search for literature sources, the following queries were used: “vasculitis”, “thrombosis”, “neutrophil extracellular traps”, “ANCA-associated vasculitis”, “venous thrombosis”, “arterial thrombosis”, “Behçet’s disease”, “Takayasu arteritis”, “granulomatosis with polyangiitis”, “eosinophilic granulomatosis with polyangiitis”, “Mmicroscopic polyangiitis”, “Henoch–Schönlein purpura”, “Kawasaki disease”, “polyarteritis nodosa”, “giant cell arteritis”, “Buerger’s disease”. In addition, use similar requests in English. The search was carried out in PubMed.

Churg-Strauss syndrome is a rare autoimmune disease with small-vessel vasculitis. We report a case of 60-year-old man. The presence of hypereosinophilia, asthma, sinusitis and extravascular eosinophil accumulation led to the diagnosis of Churg-Strauss syndrome. The patient was effectively treated with methylprednisolone, cyclophosphamide.

The description of a rare combination syndrome Churg-Strauss syndrome (CSS) with autoimmune thrombocytopenia. The disease is characterized by slow progression and refractory to standard types of immunosuppressive therapy. The first positive results are used prolonged courses of immunosuppressive of dipeptide thymodepressin

Lung damage, along with the pathology of the upper respiratory tract and kidneys, constitute the classic triad of Wegener's granulomatosis with polyangiitis (GPA). The special features of pulmonary pathology in this disease include the possibility of asymptomatic course. A case of asymptomatic lung damage in a patient with GPA has been reported. Despite clear signs of drug-induced clinical and laboratory remission, routine computed tomography (CT) revealed progressive changes in lung parenchyma as multiple foci with destruction, which regressed after treatment with cyclophosphamide. The importance of instrumental examination, including CT of the chest at the onset of GPA and during the subsequent follow-up, was emphasized.

The paper describes an autopsy case of a 69-year-old woman with microscopic polyangiitis lasting about 1.5 months despite intensive treatment. She had generalized productive vasculitis, extracapillary productive glomerulonephritis, adult respiratory distress syndrome with focal pneumonia, isolated lung atelectasis, splenic infarction, and toe and finger gangrene. Morphological differences from those of granulomatosis with polyangiitis and Churg-Strauss syndrome are presented.

Aim: To assess the significance of determining the serum and urinary concentrations of monocyte chemotactic protein-1 (MCP-1), kidney injury molecule-1 (KIM-1), and type IV collagen in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to estimate the activity of renal involvement in AAV., Subjects and Methods: 78 patients (32 men and 46 women) (median age 55 (45; 61) years) with AAV were examined. The patients were divided into 3 groups according to the AAV activity estimated using the Birmingham vasculitis activity Score (BVAS): 1) 25 patients with active ANCA-associated glomerulonephritis (GN); 2) 26 patients with active AAV without renal involvement; 3) 27 patients in sustained AAV remission. The serum and urinary concentrations of the markers were measured by enzyme immunoassay., Results: The urinary concentration of all 3 biomarkers was higher in patients with renal involvement (Group 1); the differences in the levels of MCP-1 and type IV collagen were statistically significant as compared to Groups 2 and 3 (p<0.01), while that in KIM-1 level was only in Group 2. There were statistically significant correlations between the urinary concentration of these biomarkers and the traditional GN activity indices (erythrocyturia, daily proteinuria (DPU), total BVAS scores that reflect renal involvement, as well as serum creatinine levels and estimated glomerular filtration rate (p<0.05). ROC curve analysis showed that the urinary MCP-1 excretion of ≥159 pg/ml had the highest (92%) sensitivity and urinary type IV collagen excretion of ≥3.09 µg/l had the highest (86%) specificity in assessing the activity of ANCA-associated GN. At the same time, their diagnostic value increased in terms of a combination of DPU and ESR (96% sensitivity, 84.9% specificity)., Conclusion: The urinary excretion of MCP-1, KIM-1, and type IV collagen reflects the severity of local renal inflammation in AAV patients and a study of these indicators is a promising diagnostic tool for assessing the activity of ANCA-associated GN.