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Vasovagal syncope (VVS) can occur in every third of human population. Clinical symptoms of VVS areas a result of arterial hypotension with critical global cerebral hypoperfusion due to vasodilatation and bradicardia. Bradicardia is manifested as sinus node dysfunction and atrioventricular conduction disturbances due to activation of nervus vagus. Asystole can take place in some cases. Lack of efficacy of permanent pacemaker founds in patients to prevent of VVS. The results of double blind placebo controlled studies, European and American expert's opinions, probable causes of lack of efficacy of pacemakers in such category of patients and way of solution of this problem are discussed in the review. Syncope recurrences in spite of pacemaker implantation, risk of surgery complications and good life prognosis are arguments for therapeutic approach, now suitable for the most of patients with VVS. Case report (VVS with asystole but without of pacemaker implantation) with successful follow-up is analyzed in the article.

Russian Society of Cardiology (RSC).With the participation of Russian Scientific Society of Clinical Electrophysiology, Arrhythmology and Cardiac Pacing, Russian Association of Pediatric Cardiologists, Society for Holter Monitoring and Noninvasive Electrocardiology.Approved by the Scientific and Practical Council of the Russian Ministry of Health.

Russian Society of Cardiology (RSC).With the participation of Russian Scientific Society of Clinical Electrophysiology, Arrhythmology and Cardiac Pacing, Russian Association of Cardiovascular Surgeons.Approved by the Scientific and Practical Council of the Russian Ministry of Health.

Aim. To compare autonomic nervous system activity estimated by power spectral analysis of heart rate variability in patients with vasovagal syncopes and healthy volunteers. Material and methods. Seventeen health volunteers and 25 patients with vasovagal syncope were included in the study. In 16 cases faints were induced by head-up tilt table test (HTT), in 9 cases - by bicycle exercise test (BET). Power spectral analysis of heart rate variability in 5-th min ECG records was performed in supine and upright position while breath was fixed. Results. In supine position the data of low frequency (LF) and high frequency (HF) of spectral power in both groups of patients were significantly higher than in volunteers. During upright the HF of spectral power significantly decreased in patients and volunteers. In upright position LF of spectral power significantly decreased in patients with syncope induced by HTT, did not change in patients with syncope induced by BET, but significantly increased in volunteers. Conclusion. Patients with vasovagal syncope had abnormal autonomic nervous system activity: a high level of parasympathetic influence in supine position and poor reaction of sympathetic nervous system during upright.

Aim. To study efficiency of bicycle exercise test and long-term orthostatic test in differential diagnosis of syncopes. Material and methods. The study included 113 patients suffering from recurrent syncopes of unclear origin. Twelve healthy volunteers were selected for assessment of the tests specificity. All the patients and controls were examined by means of long-term orthostatic test (LTOT) and bicycle exercise test (BET). Results. Specificity of both tests was 100%,. In LTOT syncopes were observed in 52(46%) cases, including vasovagal syncopes (51 patients), epileptic seizure (1 patient). BET induced vasovagal syncopes in 54(48%) patients. The results of both tests by inducing syncopes coincided in 30 (26%) cases. In 24(21%o) patients syncopes occurred only in BET while LTOT appeared uninformative. In 21 (18.6%) cases syncopes occurred only in LTOT while BET produced a negative result. 38 (34%) patients had syncopes neither in LTOT nor BET. Both tests induced syncopes in 75 (66%) patients. No complications were observed. Conclusion. LTOT and BET are safe and complementary methods in differential diagnosis of syncopes.

Drug-induced hypersensitivity syndrome (DIHS) or drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially fatal systemic reaction characterized by multiorgan damage involving the liver, hematopoietic system and skin, and heterogeneous manifestations of fever, rash, lymphadenopathy and eosinophilia with unpredictable course.We describe a 41-year-old female patient who developed DRESS syndrome after taking sulfasalazine prescribed for non-radiographic axial spondyloarthritis. Treatment with intravenous and then oral glucocorticoids was effective. A review of the literature on this topic is presented.

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.

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.