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<article article-type="research-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="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">rsp</journal-id><journal-title-group><journal-title xml:lang="ru">Научно-практическая ревматология</journal-title><trans-title-group xml:lang="en"><trans-title>Rheumatology Science and Practice</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1995-4484</issn><issn pub-type="epub">1995-4492</issn><publisher><publisher-name>IMA-PRESS, LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.47360/1995-4484-2021-643-663</article-id><article-id custom-type="elpub" pub-id-type="custom">rsp-3096</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="ru"><subject>ПЕРЕДОВАЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>LEADING ARTICLE</subject></subj-group></article-categories><title-group><article-title>Болезнь Стилла взрослых: новые горизонты</article-title><trans-title-group xml:lang="en"><trans-title>Adult Still's disease: New horizons</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1598-8360</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Насонов</surname><given-names>Е. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Nasonov</surname><given-names>E. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Насонов Евгений Львович.</p><p>115522, Москва, Каширское шоссе, 34а; 119991, Москва, ул. Трубецкая, 8, стр. 2.</p></bio><bio xml:lang="en"><p>Evgeny L. Nasonov.</p><p>115522, Moscow, Kashirskoye Highway, 34A; 119991, Moscow, Trubetskaya str., 8, building 2.</p></bio><email xlink:type="simple">nasonov@irramn.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9872-5282</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Файст</surname><given-names>Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Feist</surname><given-names>E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>39245, Гоммерн, Софи-в.-Беттихер-Штрассе, 1.</p></bio><bio xml:lang="en"><p>Eugen Feist.</p><p>39245, Gommern, Sophie-v.-Boetticher-Straße, 1.</p></bio><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Научно-исследовательский институт ревматологии им. В.А. Насоновой; Первый Московский государственный медицинский университет имени И.М. Сеченова Минздрава России (Сеченовский Университет)</institution><country>Россия</country></aff><aff xml:lang="en"><institution>V.A. Nasonova Research Institute of Rheumatology; I.M. Sechenov First Moscow State Medical University of the Ministry of Health Care of Russian Federation (Sechenov University)</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Отделение ревматологии Клиники Гелиос Фогельсанг-Гоммерн, партнер по сотрудничеству Магдебургского университета имени Отто фон Герике</institution><country>Германия</country></aff><aff xml:lang="en"><institution>Department of Rheumatology, Helios Clinic Vogelsang-Gommern, cooperation partner of the Otto-von-Guericke University Magdeburg</institution><country>Germany</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2021</year></pub-date><pub-date pub-type="epub"><day>26</day><month>12</month><year>2021</year></pub-date><volume>59</volume><issue>6</issue><fpage>645</fpage><lpage>665</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Насонов Е.Л., Файст Е., 2021</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="ru">Насонов Е.Л., Файст Е.</copyright-holder><copyright-holder xml:lang="en">Nasonov E.L., Feist E.</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://rsp.mediar-press.net/rsp/article/view/3096">https://rsp.mediar-press.net/rsp/article/view/3096</self-uri><abstract><p>Болезни Стилла у детей (системный ювенильный идиопатический артрит (ЮИА)) и у взрослых (болезнь Стилла взрослых (БСВ)) рассматриваются как системные аутовоспалительные заболевания неизвестной этиологии, в основе которых лежат сходные иммунопатогенетические механизмы, связанные с генетически детерминированными нарушениями механизмов врожденного иммунитета. БСВ впервые была описана 50 лет назад английским ревматологом Эриком Байуотерсом (Eric George Lapthorne Bywaters). Молекулярную основу иммунопатогенеза БСВ составляет активация врожденного иммунитета, связанная с NLRP3 инфламмасома-зависимыми механизмами воспаления, характеризуется гиперпродукцией «прово-спалительных» цитокинов — интерлейкина (ИЛ) 1 и ИЛ-18, — индуцирующих синтез других провоспалительных медиаторов воспаления. Представлен обзор новых данных, касающихся механизмов иммунопатологии, клинического полиморфизма, лабораторных биомаркеров и возможностей фармакотерапии БСВ.</p><p>Особое внимание уделено перспективам применения моноклональных антител к ИЛ-1β — канакинумаба. Рассматриваются проблемы, связанные с общностью клинико-лабораторных нарушений, патогенетических механизмов и фармакотерапии БСВ и коронавирусной болезни 2019 (COVID-19).</p></abstract><trans-abstract xml:lang="en"><p>Still's disease in children (systemic juvenile idiopathic arthritis - JIA) and adult Still's disease (ASD) are considered as systemic autoinflammatory diseases of unknown etiology, which are based on similar immunopathogenetic mechanisms associated with genetically determined disorders of the mechanisms of innate immunity. ASD was first described 50 years ago by the English rheumatologist Eric George Lapthorne Bywaters. The molecular basis of ASD immunopathogenesis is the activation of innate immunity associated with NLRP3 inflammasome-dependent mechanisms of inflammation, characterized by the overproduction of “pro-inflammatory” cytokines - interleukin (IL) 1 and IL-18, inducing the synthesis of other proinflammatory inflammatory mediators. A review of new data concerning the mechanisms of immunopathology, clinical polymorphism, laboratory biomarkers and the possibilities of ASD pharmacotherapy is presented.</p><p>Particular attention is paid to the prospects for the use of monoclonal antibodies to IL-1β - canakinumab. The problems associated with the generality of clinical and laboratory disorders, pathogenetic mechanisms and pharmacotherapy of ASD and coronavirus disease 2019 (COVID-19) are considered.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>болезнь Стилла взрослых</kwd><kwd>интерлейкин 1</kwd><kwd>COVID-19</kwd><kwd>канакинумаб</kwd><kwd>генно-инженерные биологические препараты</kwd></kwd-group><kwd-group xml:lang="en"><kwd>adult Still's disease</kwd><kwd>interleukin 1</kwd><kwd>COVID-19</kwd><kwd>canakinumab</kwd><kwd>biologics</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">McGonagle D, McDermott MF. a proposed classification of the immunological diseases. PLoS Med. 2006;3:e297. doi: 10.1371/journal.pmed.0030297</mixed-citation><mixed-citation xml:lang="en">McGonagle D, McDermott MF. a proposed classification of the immunological diseases. 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