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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">10.14412/1995-4484-2018-5-18</article-id><article-id custom-type="elpub" pub-id-type="custom">rsp-2685</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>ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL RESEARCH</subject></subj-group></article-categories><title-group><article-title>Развитие учения об аутовоспалительных заболеваниях в XXI в.</article-title><trans-title-group xml:lang="en"><trans-title>Development of the doctrine of auto-inflammatory diseases in the XXI century</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Фёдоров</surname><given-names>Е. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Fedorov</surname><given-names>E. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва, 115522 Москва, Каширское шоссе, 34А</p></bio><bio xml:lang="en"><p>134A, Kashirskoe Shosse, Moscow 115522</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Салугина</surname><given-names>С. О.</given-names></name><name name-style="western" xml:lang="en"><surname>Salugina</surname><given-names>S. O.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва, 115522 Москва, Каширское шоссе, 34А</p></bio><bio xml:lang="en"><p>134A, Kashirskoe Shosse, Moscow 115522</p></bio><email xlink:type="simple">pafon1@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кузьмина</surname><given-names>Н. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Kuzmina</surname><given-names>N. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Москва, 115522 Москва, Каширское шоссе, 34А</p></bio><bio xml:lang="en"><p>134A, Kashirskoe Shosse, Moscow 115522</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>V.A. Nasonova Research Institute of Rheumatology</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2018</year></pub-date><pub-date pub-type="epub"><day>03</day><month>04</month><year>2019</year></pub-date><volume>56</volume><issue>0</issue><issue-title>приложение 4</issue-title><fpage>5</fpage><lpage>18</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Фёдоров Е.С., Салугина С.О., Кузьмина Н.Н., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Фёдоров Е.С., Салугина С.О., Кузьмина Н.Н.</copyright-holder><copyright-holder xml:lang="en">Fedorov E.S., Salugina S.O., Kuzmina N.N.</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/2685">https://rsp.mediar-press.net/rsp/article/view/2685</self-uri><abstract><p>В статье представлено развитие учения об аутовоспалительных заболеваниях (АВЗ) в последнее десятилетие. Изложены сведения об интерлейкине 1, инфламмасомах и их роли в развитии АВЗ, а также об интерферонопатиях и, в частности, протеасомных болезнях как новом классе аутовоспалительной патологии. Показано многообразие вариантов АВЗ, включая нозологии с доминированием поражения одной системы (кожа, кишечник, кости). Обсуждаются достижения в диагностике и оценке течения АВЗ. Показана взаимосвязь АВЗ с проблемами фундаментальной и клинической ревматологии.</p></abstract><trans-abstract xml:lang="en"><p>The article presents the development of the doctrine of autoinflammatory diseases (AID) in the last decade. Data on interleukin 1, inflammasomes and their role in the development of AID are presented. The paper also contains the data about interferonopathies and, in particular, proteasomal illnesses as a new class of hereditary autoinflammatory diseases. Variety of AID variants, including diseases with the dominance of one system damage (skin, intestines, bones) is shown. Advances in the AID diagnosis and course assessment of are discussed. Relationship of the AID with the problems of fundamental and clinical rheumatology is demonstrated.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>аутовоспаление</kwd><kwd>интерлейкин 1</kwd><kwd>инфламмасомы</kwd><kwd>протеасомы</kwd><kwd>интерферонопатии</kwd></kwd-group><kwd-group xml:lang="en"><kwd>autoinflammation</kwd><kwd>interleukin 1</kwd><kwd>inflammasomes</kwd><kwd>proteasomes</kwd><kwd>interferonopathies</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">Кузьмина НН, Салугина СО, Федоров ЕС. Аутовоспалительные заболевания и синдромы. Учебнометодическое пособие. Москва: ИМА-ПРЕСС; 2012.</mixed-citation><mixed-citation xml:lang="en">Kuz'mina NN, Salugina SO, Fedorov ES. Autovospalitel'nye zabolevaniya i sindromy. Uchebno-metodicheskoe posobie [Autoinflammatory diseases and syndromes. 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