TAFRO syndrome associated with C3 nephropathy (an analysis of clinical experience)

Aim. To present a clinical case of TAFRO syndrome – a recently described subtype of idiopathic multicentric Castleman disease of unknown etiology, characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis of the bone marrow and / or renal dysfunction, organomegaly associated with an increase in serum IL-6 secretion.Materials and methods. Herein, we report a young patient diagnosed with TAFRO combined with C3 nephropathy. The patient was treated with glucocorticoids (methylprednisolone), colchicine, sarilumab, rivaroxaban, and symptomatic treatment.Results. Key aspects of diagnosis, treatment and course of the syndrome were discussed. The possibility of the combined course of two rare pathologies – a subtype of idiopathic multicentric Castleman disease and C3 nephropathy is still unclear.Conclusion. The diagnosis of TAFRO syndrome can be considered after the exclusion of a number of infectious, paraneoplastic, systemic connective tissue diseases, POEMS syndrome, liver cirrhosis against the background of autoimmune hepatitis, autoimmune thrombocytopenic purpura, as well as hemolytic-uremic syndrome. Kidney damage in this category of patients, in most of the described clinical cases, is morphologically represented by thrombotic microangiopathy or mesangioproliferative glomerulonephritis. C3 nephropathy in that clinical case seems to be concomitant. Glucocorticoids in high and ultrahigh doses, cyclosporine, IL-6 type inhibitors, as well as anti-CD20 antibodies are used as therapy

Difficult-to-treat rheumatoid arthritis: predictors and prevention possibilities (analysis of the Moscow unified register of arthritis data)

Aim. To search for factors associated with the occurrence of a difficult-to-treat state, reflecting the characteristics of both the patient and his disease, as well as preceding treatment.   Materials and methods. The analysis includes data of patients with rheumatoid arthritis (RA) from the Moscow Unified Arthritis Registry (MUAR). All included patients received вiological Disease-Modifying Antirheumatic Drugs (bDMARDs) and / or targeted synthetic DMARDs (tsDMARDs). Prior to this, all patients were treated with tsDMARDs. The group of difficult-to-treat patients included persons in whom attempts to treat bDMARDs / tsDMARDs with the first two classes of drugs were not successful, and attempts to use bDMARDs / tsDMARDs were continued. Patients in whom an attempt to treat bDMARDs / tsDMARDs of the first or second class proved effective and treatment with the appropriate drug lasted at least 2 years are not difficult-to-treat. All other cases were excluded from the analysis. The differences between the two groups were studied according to various indicators, including demographic, anthropometric, indicators describing the features of the disease, clinical manifestations of the onset and further course of the disease. From the indicators that demonstrated significant differences between the groups, significant independent predictors of difficult-to-treatstate were identified within the linear-logistic regression model by forward stepwise selection. Data describing preceding treatment were analyzed within the framework of a multifactorial linear logistic model with included selected independent predictors of achieving difficult-to-treat state (resistance to treatment).   Results. In the study were included 1044 patients, including 112 patients (10.7 %) were classified as difficult-to-treat. As significant independent predictors of treatment resistance, the following were identified: a patient’s message about an increase in body temperature at the onset of the disease of more than 38°C (p < 0.001); a patient’s message about swelling of the hands during the disease (p = 0.033); the presence of rheumatoid nodules (p = 0.022); a relatively short time since the appearance of joint pain till contacting a doctor (p = 0.021). A significant relationship was found betweenbDMARD / tsDMARD used as first-line drug and the subsequent categorization of the patient as difficult-to-treat (p = 0.013). In patients receiving tofacitinib or rituximab in the first line of targeted therapy, the risk of subsequent treatment resistance was significantly lower than in patients receiving tumor necrosis factor inhibitors (iFNO): relative risk (RR) 0.017, 95 % confidence interval (CI) 0.04–0.72) and RR 0.47, 95 % CI 0.22–0.98), respectively. The analysis of the second line of targeted therapy revealed a significant advantage of etanercept over some other classes of bDMARDs / tsDMARDs in terms of the risk of subsequent treatment resistance. Thus, RR for other drugs of the iFNO class was 2.48 (95 % CI 1.13–5.44, p = 0.023); for abatacept – 3.15 (95 % CI 1.488–6.68, p = 0.003), and for tocilizumab – 2.57 (95 % CI 1.03–6.40; p = 0.043).   Conclusion. The analysis of data from the registry of patients who were in real clinical practice made it possible to identify patients who accurately meet criteria of the European Alliance of Associations for Rheumatology for difficult-to-treat RA and patients who exactly do not meet them. A comparative analysis of the groups revealed predictors of following classifying the patient as difficult-to-treat. Based on the listed signs, high-risk groups of resistance to treatment with bDMARDs / tsDMARDs can be identified. The results obtained give grounds for the preferred use in such patients on the first line of treatment of tofacitinib or, possibly, other JAK kinase inhibitors, and on the second line of etanercept

Eternal Middle Ages: Time Models in Modern Mass Culture

Eternal Middle Ages: Time Models in Modern Mass Culture (by Aleksey Pozharov). Nowadays in the works of mass culture, a remarkable interest in the Middle Ages is noticed. According to many cultural studies, it is not so much the historical Middle Ages reflected in the cultural artifacts as it is the contemporary society set in medieval surroundings. What images and ideas does the modern mass culture deliver? In order to deal with this issue, it’s necessary to consider one of the fundamental concerns of civilization: which model of time is relevant to the contemporary cultural artifacts.</jats:p

Institutional Study of the European Union Intelligence and Situation Centre (EU INTCEN)

This article delves into the intricate domain of the European Union's intelligence apparatus, notably, the European Union Intelligence and Situation Centre (EU INTCEN). Employing a historiographical analysis of both domestic and international literature, the authors synthesize insights from Russian and foreign scholars concerning the genesis of intelligence operations in a unified Europe, focusing on their structural attributes. Furthermore, this study seeks to evaluate the contemporary state and potential directions of the EU's intelligence activities, considering both their functional and institutional dimensions

Giant Cell Arteritis in Ophthalmology Practice: a Case Report

Introduction. Giant cell (temporal) arteritis refers to a group of chronic and acute systemic vasculitis mainly affecting the extracranial and intracranial arteries of large and medium caliber. Loss of vision due to anterior ischemic optic neuropathy (AION) or occlusion of the central retinal artery is one of the most severe and most common complications of giant cell arteritis. This case report describes a patient with giant cell arteritis, the outcome of it was a total vision loss in the right eye. The patient repeatedly visited the ophthalmologists in the outpatient clinics with complaints of intermittent episodes of vision loss.Purpose. To present methods of diagnosis and treatment, through which the doctor at the initial reception will be able to suspect the disease and start treatment timely.Conclusions. The anamnestic criteria for the GCA diagnosis in the practice of ophthalmologist are: female, age over 50 years, headaches with paresthesia, intermittent lameness of the mandible, short-term episodes of vision loss. The necessary laboratory methods of research include: clinical blood test with determination of erythrocyte sedimentation rate, determination of C-reactive protein level.</jats:p