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The aim – to present the experience of diagnosis, management, and therapy with IL-1 inhibitors in patients with Schnitzler’s syndrome (SchS) according to a multicenter Russian cohort.Materials and methods. In an observational retrospective study for a 10-year period (2012–2022), 17 patients with SchS who were admitted to the hospital or were observed on an outpatient basis, among them 8 women, 9 men, were included in the study. The diagnosis of all corresponded to the Strasbourg diagnostic criteria.Results. The age of patients ranged from 25 to 81 years (Me 53 [46; 56] years). The age at the time of the onset of the disease ranged from 20 to 72 years (Me 46 [39; 54] years), the duration of the disease before diagnosis ranged from 1 to 35 years (Me 6.5 [3; 6] years), in 3 it exceeded 10 years, in the rest it ranged from 1 to 8 years. Infectious and lymphoproliferative diseases, monogenic AIDS (CAPS, TRAPS, HIDS) were excluded from all patients at the prehospital stage. The guiding diagnosis for all was Still’s disease in adults. Clinical manifestations of the disease included: fatigue, lethargy, fatigue, rash and fever in all, skin elements were urticular in all, accompanied by itching in 6 (37.5%). Bone pain had 12 (70.6%), arthralgia – 16 (94.1%), arthritis – 9 (52.9%), myalgia – 7 (41.2%), weight loss in 4 (23.5%). Lymphadenopathy (6), enlarged liver (6), pericarditis (4), angioedema (6), redness and dryness in the eyes (3), sore throat (2), abdominal pain (1), distal polyneuropathy (2), paraesthesia (1), chondritis of the auricles were less common (1). Monoclonal gammopathy was detected in all with a secretion level of 2.9–15.1 g/l: IgMk (n=10 (64.7%)), less often IgMλ (n=2), IgGk (n=2), IgGλ (n=1), IgAλ (n=1). Ben-Jones protein was not detected in any of them. All patients had an increase in the level of ESR, CRP. 16 patients before inclusion in the study received GC (94.1%) with a temporary effect and its escape with dose reduction or cancellation, DMARD – 7, among them methotrexate (5), hydroxychloroquine (2), cyclophosphamide (1), also NSAIDs and antihistamines in all, biological drugs: anti-B-cell the drug rituximab (1), monoclonal AT to IgE – omalizumab in 2 (1 – without effect, 1 – partial effect). 11 patients were prescribed IL-1: canakinumab – 9 (52.9%) subcutaneously once every 8 weeks, anakinra – 4 (23.5%) subcutaneously daily. The duration of taking anakinra, which was prescribed in the test mode, ranged from 1 week to 2.5 months with a further switch to canakinumab in 3. The duration of taking canakinumab at the time of analysis ranged from 7 months to 8 years. Against the background of treatment with IL-1, 10 out of 11 (90.9%) received a complete response from the clinical manifestations of the disease and a decrease in the level of ESR and CRP within a few days. In 1 patient, a partial response was received to the administration of anakinra, and when switching to canakinumab, the effect of treatment was finally lost. 1 patient received IL-6 for 8 months with incomplete effect and transition to IL-1 with positive dynamics. In 1 patient, due to the persistent absence of relapses, the interval between canakinumab injections was increased to 5 months without signs of reactivation, but subsequently, against the background of stress and relapses of the disease, the intervals were reduced to 4 months. A healthy child was born in the same patient on the background of treatment. The tolerability of therapy was satisfactory in all patients, no SAE was noted.Conclusion. SchS is a rare multifactorial/non–monogenic AID that needs to be differentiated from a number of rheumatic diseases and other AIDS. The onset in adulthood, the presence of recurrent urticarial rashes in combination with fever and other manifestations of a systemic inflammatory response are indications for examination for monoclonal secretion. The use of short- or long-acting IL-1 is a highly effective and safe option in the treatment of such patients.

Juvenile idiopathic inflammatory myopathies (JIIM) are rare diseases in which the prognosis is largely determined by timely diagnosis, timing of prescription and effectiveness of therapy.Objective: to characterize the clinical phenotypes, the results of paraclinical examination methods, the spectrum of autoantibodies, as well as therapeutic options in patients with JIIM.Patients and methods. The retrospective study included 37 patients with JIIM hospitalized in the pediatric department of the V.A. Nasonova Research Institute of Rheumatology from 2016 to 2020. All patients underwent a standard clinical and laboratory-instrumental examination in accordance with the diagnosis and severity of the condition.Results and discussion. Twenty-three of the 37 JIIM patients had juvenile dermatomyositis (JDM), 1 had polymyositis, and 13 had overlap-myositis (OM). The ratio of boys and girls was 1:1.7. The median age of onset for JDM was 6.9 years, and OM was 11.3 years. All patients had skeletal muscles involvement, dysphagia was detected in 52.2% of cases of JDM and in 15.4% of cases of OM. An increase in the level of creatine phosphokinase at the time of diagnosis was observed in 72.9% of patients, of lactate dehydrogenase – in 81.1%, of alanine aminotransferase - in 67.6%, of aspartate aminotransferase – in 75.7%. Heliotrope rash and/or Gottron's syndrome were observed in 100% of patients with JDM and in 30.8% with OM. Lung involvement was found in 27% of children. The myopathic capillaroscopic changes were present in 95.2% of patients with JDM and 53.8% with OM. Myositis-specific autoantibodies were found in 10.8% of patients with JIIM.All patients received glucocorticoids, 81.0% methotrexate, 18.9% hydroxychloroquine, 8.1% cyclophosphamide, 8.1% cyclosporine, 2.7% mycophenolate mofetil, 2.7% azathioprine, 67.6% - intravenous immunoglobulin. Biologic disease modifying antirheumatic drugs (bDMARDs) were prescribed to 26% of patients with JDM and to all patients with OM (only in 51.3% of cases with JIIM). The median duration of illness before initiation of bDMARDs therapy was 2.25 years. 58.8% of patients were treated with rituximab (RTM), 41.2% with abatacept (ABA). In 1 patient with OM, represented by a combination of systemic lupus erythematosus and JDM, three bDMARDs were used sequentially: ABA, etanercept and RTM. All patients achieved inactive disease status.Conclusion. JDM is the most common phenotype of JIIM, which is characterized by an earlier age of onset, skin involvement that precedes the development of myopathy, and typical capillaroscopic changes. The conclusion was made about the high efficacy and acceptable safety profile of bDMARDs therapy in children with JIIM, with careful monitoring of its tolerability. bDMARDs can be prescribed even in the early stages of the disease in the presence of unfavorable prognostic factors.

Ferritin is a complex protein composite (iron protein) that plays the role of the main intracellular iron depot in humans and animals, consisting of the protein apoferritin and the ferric atom in the composition of phosphate hydroxide. The reference value of ferritin in women is 200 μg/l, in men – 300 μg/l. Ferritin is a marker of total body iron stores, and low levels are specific for iron deficiency. Ferritin is also involved in immune processes and has both pro-inflammatory and immunosuppressive activity. Hyperferritinemia is a nonspecific symptom that occurs in a number of immunoinflammatory, infectious diseases, as well as during body iron stores overload. Hyperferritinemia is a criterion sign of macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis, systemic lupus erythematosus and Kawasaki disease, as well as a predictive biomarker of adult-onset Still's disease. High ferritin levels occur in catastrophic antiphospholipid syndrome, as well as in infectious pathologies such as septic shock and COVID-19, including multisystem inflammatory syndrome associated with COVID-19. Ferritin concentration is an important parameter for assessing the activity and prognosis of the disease, which allows a rational approach to the choice of therapy in these patients.

Autoinflammatory diseases (AIDs) are a heterogeneous group of rare genetically determined conditions, the main manifestations of which are episodes of fever in combination with other signs of systemic inflammation: skin rashes, musculoskeletal and neurological disorders, damage to the organs of vision, hearing, etc., as well as acute phase markers and the absence of autoantibodies. The use of biological therapy, especially inhibitors of interleukin 1 (iIL1), in most common monogenic AIDs (mAID) – FMF, TRAPS, HIDS/MKD, CAPS – has shown its high efficiency and led to significant progress in the treatment of these patients. Currently, iIL1 are the first-line drugs for mAIDs therapy, primarily CAPS. In the case of their ineffectiveness or intolerance in certain situations, other biologic disease-modifying antirheumatic drugs can also be used – inhibitors of tumor necrosis factor α and iIL6, but this issue needs further investigation. The article describes a patient with mAID, in whom the diagnosis was made more than 40 years after the onset; administration of targeted therapy even in the late stages of the disease led to a significant improvement in many symptoms and quality of life.

In recent years, increasing attention has been paid to the problem of preserving the genetic diversity of farm animals and poultry. However, an indispensable condition for the economic feasibility of preserving any breed is not only its uniqueness, but also the possibility of practical use. An example is the Russian snow-white breed of chickens in the VNIIGRZh bioresource collection. It was developed by breeding of Russian white chickens for thermal resistance at low temperature conditions, as well as for resistance to neoplastic diseases. Currently, this breed is specialized for the purposes of the bio-industry (raw materials for the production of embryonic viral vaccines). The change in the direction of breeding work led to a modification of the genetic structure of the population. Constant selective pressure on one particular trait can lead to a decrease in variability around the genomic regions associated with that trait. Therefore, understanding the genetic mechanisms leading to phenotypic differentiation requires the identification of regions in the genome that were under selection pressure. The study of runs of homozygosity (ROH) rates can provide useful information about the history of population selection, as well as allow for a better understanding of the genotype-phenotype relationship by discovering genes that are or were under selection pressure. In ROH islands specific to two generations of chickens, the most relevant candidate genes associated with adaptive traits are annotated. It has been established that genetic analysis based on changes in ROH can be used to characterize the genetic profile of chickens and to change the structure of the population under the influence of selection pressure. These data are especially important to take into account when evaluating qualitative phenotypic features, such as the adaptive capabilities of chickens.

The article is devoted to the problem of increasing the resistance of chicks from hatching to the age of 3 weeks to infectious diseases by technological methods. Dosed low-temperature exposure to the embryo during the sensitive period of early embryogenesis was proposed as an impact factor; influenza vaccine virus was used as a test virus for experimental infection of embryos. It was found that in the embryos after cooling, the titer of the virus was significantly lower by 1.5-12.0 times (p

The article is devoted to the life and various aspects of the activities of the outstanding diplomat Prince Alexey B. Lobanov-Rostovsky (1824–1896). The name of Lobanov-Rostovsky was not mentioned in literature until the first decade of the 21st century. Then Lobanov began to be remembered thanks to the efforts of his descendant Prince Nikita D. Lobanov-Rostovsky who initiated a number of studies about the prominent diplomat. A fundamental monograph on Prince Lobanov-Rostovsky has now been prepared. His international activity as Ambassador Extraordinary and Plenipotentiary took place at the most difficult political points at that time — twice in Constantinople (1859–1863; 1878–1879), as well as in London (1879–1882) and Vienna (1882–1895). He always managed to solve the most pressing problems of his time using skillful diplomatic tools. He received the post of Deputy Minister of the Ministry of Internal Affairs during the challenging period of restructuring of social and public institutions in Russia. It was the era of the so called Great Reforms of Emperor Alexander II. By offering him this position, the emperor took into account Prince’s intellectual scale, strategic foresight and tactical flexibility. During 11 years of activity in this field (1867–1878), Prince Lobanov-Rostovsky often performed the duties of a minister. During this time, the legal norms of the state and its relations with society significantly improved. In 1870, Alexey B. Lobanov-Rostovsky received the honorary position of State-Secretary of His Imperial Majesty, that is, the emperor's personal speaker, the position he held until the end of his life. Both Emperor Alexander II and Nicholas II treated Lobanov-Rostovsky with special warmth. Lobanov-Rostovsky managed to conclude the Russian-Turkish final Peace Treaty of 1879, which put an end to the war between Russia and Turkey. This agreement gave Russia significant moral and material results and marked the beginning of the autonomous Principality of Bulgaria, that led to Bulgarian statehood. Lobanov-Rostovsky’s work as Foreign Minister for 18 months (1895–1896) brought Russia fruitful results on the world stage and was highly appreciated both in Russia and in the world. The article talks about the different sides of the prince’s extraordinary personality. A true polymath, proficient in the Russian antiquity, he became a bibliophile, collector, genealogist and historian. He carefully commented on the historical documents he discovered and for 14 years (1871–1885) systematically published in special historical journals. The article examines the memoirs of contemporaries about the diplomat, as well as the latest research about him.

Taking these prospects into account, the integration potential of Russia in the conditions of gradual trade and economic unity of the ASEAN countries can be considered in the aspect of formation of new strategic opportunities for interaction with the integration environment of Eurasia on the basis of investments in infrastructure development within the framework of the Eurasian Economic Union (EAEU). The advantages and disadvantages of the digital platforms and the characteristic features of international DLPs are highlighted. Based on the analysis, the necessity of developing an international digital platform in the EAEU is justified in the context of the development of international digital logistics platforms within the framework of the Eurasian Economic Union.