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<article article-type="review-article" dtd-version="1.3" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">nnp</journal-id><journal-title-group><journal-title xml:lang="en">Neurology, Neuropsychiatry, Psychosomatics</journal-title><trans-title-group xml:lang="ru"><trans-title>Неврология, нейропсихиатрия, психосоматика</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2074-2711</issn><issn pub-type="epub">2310-1342</issn><publisher><publisher-name>"IMA-Press", LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.14412/2074-2711-2023-6-135-141</article-id><article-id custom-type="elpub" pub-id-type="custom">nnp-2150</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEWS</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОБЗОРЫ</subject></subj-group></article-categories><title-group><article-title>Neuromyelitis optica spectrum disorders and complement system: issues of pathogenesis and therapeutic options</article-title><trans-title-group xml:lang="ru"><trans-title>Заболевания спектра оптиконевромиелита и система комплемента: вопросы патогенеза и терапевтические возможности</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9769-447X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Краснов</surname><given-names>В. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Krasnov</surname><given-names>V. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Владимир Сергеевич Краснов</p><p>197022, Санкт-Петербург, ул. Льва Толстого, 6–8</p></bio><bio xml:lang="en"><p>Vladimir Sergeevich Krasnov</p><p>6-8, L’va Tolstogo St., St. Petersburg 197022</p></bio><email xlink:type="simple">krasnov_volod@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Acad. I.P. Pavlov First Saint Petersburg State Medical University of Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2023</year></pub-date><pub-date pub-type="epub"><day>17</day><month>12</month><year>2023</year></pub-date><volume>15</volume><issue>6</issue><fpage>135</fpage><lpage>141</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Krasnov V.S., 2023</copyright-statement><copyright-year>2023</copyright-year><copyright-holder xml:lang="ru">Краснов В.С.</copyright-holder><copyright-holder xml:lang="en">Krasnov V.S.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://nnp.ima-press.net/nnp/article/view/2150">https://nnp.ima-press.net/nnp/article/view/2150</self-uri><abstract><p>An important component of the pathogenesis of neuromyelitis optica spectrum diseases (NMOSD) with antibodies to aquaporin-4 (AQP4-IgG) is a classical pathway of complement system (CS) activation with the implementation of mechanisms of complement-mediated cytotoxicity. Eculizumab is a humanized monoclonal antibody that suppresses the final stage of CS activation and has a high affinity for its C5 component. The most important components in the pathogenesis of NMOSD with AQP4-IgG, the role of CS, the results of clinical trials with the drug eculizumab and its place in the treatment of NMOSD are discussed.</p></abstract><trans-abstract xml:lang="ru"><p>Важным звеном патогенеза заболеваний спектра оптиконевромиелита (ЗСОНМ) с антителами к аквапорину-4 (AQP4-IgG) является повышение активности системы комплемента (СК) по классическому пути с реализацией механизмов комплемент-опосредованной цитотоксичности. Экулизумаб – гуманизированное моноклональное антитело, которое подавляет терминальную стадию активации СК, обладая высокой аффинностью к ее С5-компоненту. Обсуждаются основные звенья патогенеза ЗСОНМ с AQP4IgG, роль в нем СК, результаты клинических исследований препарата экулизумаб, а также его место в терапии ЗСОНМ.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>заболевание спектра оптиконевромиелита</kwd><kwd>система комплемента: экулизумаб</kwd></kwd-group><kwd-group xml:lang="en"><kwd>neuromyelitis optica spectrum disease</kwd><kwd>complement system: eculizumab</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Статья спонсируется компанией «Генериум»</funding-statement><funding-statement xml:lang="en">This article has been supported by Generium</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Bruscolini A, Sacchetti M, La Cava M, et al. Diagnosis and management of neuromyelitis optica spectrum disorders – An update. 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