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Ярославская государственная медицинская академия Резюме Цель. Оценить показатели свертывания и индексы активности у больных системной красной волчанкой (СКВ). Материал и методы. Обследовано 86 больных СКВ (83 жен и 3 муж), 12 из них с АФС. Средний возраст 35,9±1,5 лет (от 18 до 58 лет), средняя длительность заболевания 9,8±1,4 лет. Контроль-60 здоровых добровольцев, средний возраст 37,1±4,1 лет. Оценка активности СКВ производилась в соответствии с индексами SLAM, SLEDAI и ECLAM. Результаты. У больных СКВ определялось 5-кратное (р<0,01) увеличение спонтанной агрегации тром боцитов и более чем Зх-кратное увеличение концентрации антигена фактора фон Виллебранда (ФВ:Аг). Активация тромбоцитов сочеталась со снижением агрегации тромбоцитов с коллагеном на 27% (р<0,01). Характерным признаком активации коагуляционного гемостаза было значительное увеличе ние-на 81% (р<0,01)-концентрации растворимых фибрин-мономерных комплексов (РФМК), а также повышение уровня Д-димеров у 53,3% больных. Концентрация фибриногена была повышена на 29%; показатели спонтанного фибринолиза снижены на 20%, активность антитромбина (AT) III уменьшена на 21% по сравнению с контролем. Выявлена прямая корреляционная связь между индексами активно сти и РФМК (ECLAM, г=0,51), концентрацией фибриногена (SLAM, г=0,34), уровнем Д-димеров (ECLAM, г=0,5), спонтанной агрегацией тромбоцитов (ECLAM, г=0,5) и обратная корреляция с актив ностью AT III (SLEDAI, г=-0,73). Заключение. Изменение гемостазиологических показателей при СКВ может служить предиктором раз вития тромботических нарушений и показанием к медикаментозной коррекции нарушений свёртыва ющей системы крови. Показана прямая зависимость между напряженностью свёртывающей системы крови и индексами активности СКВ.

Rheumatic diseases relate to the group of the immunoinflammatory diseases (IID), in pathogenesis of which have a value both autoimmune and autoinflammatory processes. Aim.To present the heterogeneous pathogenesis of inflammation in IID. Materials and methods.It is inspected 260 patients (pts) with IID: 242 pts with systemic autoimmune diseases (SAD): 65 systemic lupus erythematosis, 50 systemic sclerosis, 127 systemic vasculitides (SV) and 18 patients with autoinflammatory diseases (AID): 8 Behcets disease, 2 periodic disease, 5 familial cold fever, 2 idiopathic lobular panniculitis and 1 relapsing polychondritis. Is carried out a study of complement, antigen of von Willebrand factor (FW:AG), antinuclear antibodies, antibodies to DNA, anti-endothelial antibodies, antibodies to topoizomeraze I (anti-Scl-70), antineutrophilic cytoplasmic antibodies (ANCA), anticardiolipin antibodies (aCL IgG and aCL IgM), cryoglobulins, VS, CRP. Results.SAD were characterized by the synthesis of wide antibodies spectrum. As the basic serological marker at the screening it follows to consider antinuclear antibodies (75%). Practically in all groups it took place hypcomlemetemia with reduction of C3 and C4 complement. With systemic lupus erythematosis are revealed antibodies to DNA (71%), with ANCA-associated SV-ANCA (94%), aKL (14%); with SSD aScl-70 (17%). At Wegener granulomatosis ANCA are determined in 94% patients in the active stage. It is noted correlation ANCA with the index of the clinical activity of vasculitis. In the remaining SV groups ANCA were separated in the single cases. Cryoglobulins are noted in all patients with cryoglobulinemic vasculitis. aCL IgG and aCL IgM were the markers of antiphospholipid syndrome. Аnti-endothelial antibodies had significant oscillation spectrum. High indices FW:AG are noted with all above nosologic forms indicated, especially with Wegener granulomatosis and vasculitis hemorrhagic. Among the laboratory tests of inflammatory activity should be considered the determination of VS, CRP and FV:AG, which is also considered the marker of vascular wall defeat. Is given clinical characteristic and changes in the laboratory indices at AID: Conclusion.Isolation from the group IID of patients with AID serves as indication for a genetic study of this contingent with the approval of use for their treatment of biological therapy. Isolation from the group SAD patients with AID serves as indication for a genetic study of this contingent with the approval of use for their treatment of biological therapy.

Heterogeneity of immuno-inflammatory diseases pathogenesis has been revealed with high concentration of immunoglobulin E in some nosological forms, which requires further study.Aim. To analyze literature data, to present retrospective analysis and personal observations of patients with high concentration of IgE in the blood. Material and methods. In 7 patients with autoinflammatory diseases and 130 rheumatic patients, examined earlier, the analysis of IgE concentration has been carried out by radio-immune method or by immune-enzyme analysis. Results. Clinical observations of patients with derated immunoglobulin E syndrome are represented. The possibilities of genetic analysis and the connection of the derated IgE syndrome with vascular dysfunctions in rheumatic diseases are discussed. Conclusion. When making diagnosis is complicated in patients with inflammatory syndrome of unclear etiology and high IgE concentration in the blood, it is necessary to keep in mind the autoinflammatory genesis of this process. Further study of the role of the derated immunoglobulin E syndrome in pathogenesis is required.