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        • Тромботическая микроангиопатия в ревматологии: связь тромбовоспаления и аутоиммунитета - Журнал Терапевтический архив №5 Вопросы ревматологии 2020

        Тромботическая микроангиопатия в ревматологии: связь тромбовоспаления и аутоиммунитета - Журнал Терапевтический архив №5 Вопросы ревматологии 2020

        Насонов Е.Л., Решетняк Т.М., Алекберова З.С. Тромботическая микроангиопатия в ревматологии: связь тромбовоспаления и аутоиммунитета. Терапевтический архив. 2020; 92 (5): 4–14. DOI: 10.26442/00403660.2020.05.000697

        ________________________________________________

        Nasonov E.L., Reshetniak T.M., Alekberova Z.S. Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity. Therapeutic Archive. 2020; 92 (5): 4–14. DOI: 10.26442/00403660.2020.05.000697

        Тромботическая микроангиопатия в ревматологии: связь тромбовоспаления и аутоиммунитета

        Насонов Е.Л., Решетняк Т.М., Алекберова З.С. Тромботическая микроангиопатия в ревматологии: связь тромбовоспаления и аутоиммунитета. Терапевтический архив. 2020; 92 (5): 4–14. DOI: 10.26442/00403660.2020.05.000697

        ________________________________________________

        Nasonov E.L., Reshetniak T.M., Alekberova Z.S. Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity. Therapeutic Archive. 2020; 92 (5): 4–14. DOI: 10.26442/00403660.2020.05.000697

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          Тромботическая микроангиопатия в ревматологии:  связь тромбовоспаления и аутоиммунитета

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        • Аннотация
        • Список литературы
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        Аннотация
        Неконтролируемые гиперкоагуляция и воспаление («тромбовоспаление» – thromboinflammation), представляющие собой независимые, но тесно связанные и амплифицирующие друг друга патологические процессы, составляют основу патогенеза широкого спектра заболеваний и осложнений, в том числе при иммуновоспалительных (аутоиммунных) ревматических заболеваниях, с развитием потенциально смертельных повреждений внутренних органов. Одним из ярких прототипов «тромбовоспалительных» патологических состояний является тромботическая микроангиопатия. Существование тесной взаимосвязи между факторами внешней среды, генетическими дефектами свертывания крови и компонентов комплемента, воспалением и аутоиммунитетом в качестве патогенетических механизмов микротромбоза привлекает особое внимание к изучению тромботической микроангиопатии при иммуновоспалительных ревматических заболеваниях, в первую очередь системной красной волчанке, антифосфолипидном синдроме и склеродермическом почечном кризе. Эти исследования в перспективе могут иметь значение для расширения представлений о роли аутоиммунных механизмов патогенеза «критических» нарушений гемостаза при заболеваниях человека, разработки новых подходов к терапии. В последнее время привлечено внимание к применению для лечения системной красной волчанки и антифосфолипидного синдрома препарата экулизумаб, представляющего собой гуманизированные моноклональные IgG2/4k-антитела, блокирующие С5а-компонент комплемента и образование мембраноатакующего комплекса (С5b-9). Препарат зарегистрирован для лечения атипичного гемолитико-уремического синдрома, пароксизмальной ночной гемоглобинурии, а также тяжелых форм миастении и оптического нейромиелита. Дальнейшие исследования в этом направлении создадут предпосылки для улучшения прогноза не только у пациентов с орфанной патологией, но и широко распространенными заболеваниями человека.

        Ключевые слова: тромботическая микроангиопатия, системная красная волчанка, антифосфолипидный синдром, экулизумаб.

        ________________________________________________

        Uncontrolled hypercoagulation and inflammation (“thromboinflammation”), which are both independent and closely related and amplifying each other pathological processes, form the basis for pathogenesis of a wide range of diseases and complications, including immuno-inflammatory (autoimmune) rheumatic diseases, with the development of potentially fatal injuries of internal organs. Thrombotic microangiopathy is one of the most prominent prototypes of “thromboinflammatory” pathological conditions. The close link between environmental factors, hemostasis genetic defects and the complement system, inflammation and autoimmunity as pathogenetic mechanisms of microthrombosis draws particular attention to studying thrombotic microangiopathy in immuno-inflammatory rheumatic diseases, primarily systemic lupus erythematosus, antiphospholipid syndrome and scleroderma renal crisis. In future, these studies may be important for expanding the idea of the role of autoimmune mechanisms in pathogenesis of “critical” hemostasis disorders in human diseases, and for developing new approaches to therapy. Recently, special attention has been paid to the treatment of systemic lupus erythematosus and antiphospholipid syndrome with eculizumab, which is humanized monoclonal IgG2/4k antibody that blocks the complement component C5a and the membrane attack complex (C5b-9) formation, and which is registered for the treatment of atypical hemolytic uremic syndrome, paroxysmal nocturnal hemoglobinuria, as well as severe forms of myasthenia gravis and neuromyelitis optica. Further studies in this direction will create prerequisites for improving the prognosis not only in patients with orphan disorders, but also for widespread human diseases.

        Keywords: thrombotic microangiopathy, systemic lupus erythematosus, antiphospholipid syndrome, eculizumab.


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        Авторы
        Е.Л. Насонов1,2, Т.М. Решетняк1,3, З.С. Алекберова1

        1 ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой», Москва, Россия;
        2 ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
        3 ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России, Москва, Россия

        ________________________________________________

        E.L. Nasonov1,2, T.M. Reshetniak1,3, Z.S. Alekberova1

        1 Nasonova Research Institute of Rheumatology, Moscow, Russia;
        2 Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia;
        3 Russian Medical Academy of Continuous Professional Education, Moscow, Russia


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