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Systemic lupus erythematosus is an immunopathological disease which is characterized by a poor prognosis. Biologics applied in the disease treatment allow reducing the corticosteroid toxicity and controlling the disease.Purpose. To evaluate the efficacy and safety of rituximab therapy in children with systemic lupus erythematosus.Material and methods. The retrospective study included data of 48 patients with systemic lupus erythematosus treated with rituximab. Systemic lupus erythematosus was diagnosed based on the SLICC classification criteria. Patients were assessed at baseline disease status, at the time of rituximab initiation and follow-up. The indications for the rituximab were: lupus nephritis, CNS involvement, and hematological involvement resistant to the standard therapy, and in cases of severe corticosteroid toxicity.Results. During rituximab therapy the significant decrease of the SELENA–SLEDAI activity index was observed. There was a significant decrease of the level of antibodies against dsDNA, normalization of the levels of hemoglobin, ESR, complement C4. The proportion of patients with cytopenia decreased up to their complete absence in patients receiving therapy for three years. The number of patients with active lupus nephritis decreased from 16 at the time of rituximab initiation to 1 after 3 years of therapy. Significant dynamics of proteinuria and hematuria was noted, except for 1 patient. The daily dose of corticosteroids was reduced by 90% from baseline in patients treated for 3 years. Serious adverse events included three deaths in patients with high systemic lupus erythematosus activity with uncontrolled macrophage activation syndrome associated with infections. Various infectious complications, hypogammaglobulinemia, which required replacement therapy with intravenous immunoglobulin, were also recorded.Conclusion. Rituximab can be considered as an option for the treatment of severe forms of systemic lupus erythematosus which are resistant to standard therapy. Further studies are required to evaluate efficacy and safety.

Behcet disease is autoimmune vasculitis with predominantly mucocutaneous manifestations; eye, gastrointestinal and nervous system involvement is also possible. Behcet disease is relatively rare in pediatric population.This article presents the case of Behcet’s disease in a 17-year-old boy, with a usual delay in the diagnosis, the absence of internal organs involvement and successful first-line therapy (colchicine).

The paper presents data on the pathogenesis of systemic lupus erythematosus (SLE), and depicts various molecular mechanisms for the development of SLE and lupus-like syndromes. It describes groups of diseases, such as apoptotic defects; NETosis; interferonopathies; complement deficiency; autotolerance disorders associated with mutations in the RAG1/RAG2 genes; hereditary metabolic diseases (prolidase deficiency, deficiency of adenosine deaminase 2; lysinuric protein intolerance; and α-mannosidase deficiency). The table summarizes clinical data on most of the known lupus-like syndromes and their molecular mechanisms.The pathogenesis of many forms of monogenic lupus-like diseases is being studied. The main sign suggesting in favor of the possible monogenic disease in a patient with SLE is its onset in infancy, especially in males. Attention should be also paid to a compromised family history, including to the marriage between close relatives, the resistance of disease to standard therapy, as well as atypical symptoms.

Vasovagal syncope is the most common cause for loss of consciousness. The need for treatment of this condition is warranted by impaired quality of life and a risk of trauma due to the fall. At present, there are no medications with proven preventive effectiveness for vasovagal syncope as demonstrated by large randomized, placebo-controlled studies. At the same time, information about the pathogenesis of vasovagal reaction and provoking factors allows effective use of non-drug methods (such as patient education, physical exercise, maneuvers) in the management of patients with vasovagal syncope.

Type 1 interferons (IFN1) are both key molecules of antiviral defense and potent inflammatory mediators. In 2003, increased expression of a variety of interferon 1-regulated genes was observed in a blood cells of patients with systemic lupus erythematosus (SLE). This phenomenon was called the type 1 interferon signature (IFN1-signature). Since then, expression patterns indicating the presence of an IFN1-signature were consistently detected in a range of monogenic and complex autoimmune and autoinflammatory conditions. A quantitative indicator reflecting the degree of hyperactivation of the IFN1 pathway is known as interferon score. This review discusses the possible causes of upregulated expression of interferon 1-induced genes, the laboratory approaches to the interferon score analysis, as well as the practical use of this indicator for the diagnosis of various conditions.

The study of deformation of solids by high-pressure liquid (hydrostatic extrusion) is not only an interesting research problem, but an applied one. Hydrostatic extrusion method is currently widely used in various sectors of industry – from mechanical engineering to the food industry. This field of research seems relevant and promising. The paper presents the calculation results for some parameters of continuous hydrostatic extrusion of materials, shows graphs of the dependence of the axial stress in the billet on the total pressure drop along the length and graphs of the dependence of the total pressure drop along the length on the working fluid flow rate in the annular gap. The calculations were performed for the adiabatic fluid flow and for the flow with partial heat transfer. Analysis of the calculation results showed that for fixed flow rates of the working fluid and specified heat transfer conditions, maximum axial stresses can be obtained by optimal values taken for the ratios of the working chamber and the billet diameters.

Aim : to perform retrospective evaluation of tocilizumab (TCZ) treatment every 2 and 4 weeks. Patients and methods : 33 children with systemic juvenile idiopathic arthritis (sJIA) were observed. Results : children, who need TCZ treatment every 2 weeks had more severe sJIA course. Patients which were treated every 4 weeks had higher TCZ efficacy, no new cases of macrophage activation syndrome (MAS), lower frequency of organ involvement and relapses during TCZ treatment. In 5 (20,8%) children of this group the TCZ-free remission was achieved, and in 3/5 children total drug-free remission lasted the maximum 1085 days was reached. Only 1 children in 4 weeks group developed relapse which leaded to re-start of TCZ treatment with the same efficacy as at first. No patients, who were treated every 2 weeks experienced TCZ-free remission. Also 4 cases of MAS were detected in children who had MAS before the start of TCZ. In 3/4 TCZ was discontinued but in 1 MAS TCZ accompanied with corticosteroids was prolonged. No new MAS cases were detected during this study. Infusion reactions lead to TCZ discontinuation were in 9,1%. 1 death (3,0%) during the trial. Conclusions : we offered the set of clinical and laboratorial criteria of high and low risk patients who need TCZ treatment every 2 and 4 weeks consequently. Key words : systemic juvenile idiopathic arthritis, interleukine-6, tocilizumab.