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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">medalphabet</journal-id><journal-title-group><journal-title xml:lang="ru">Медицинский алфавит</journal-title><trans-title-group xml:lang="en"><trans-title>Medical alphabet</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2078-5631</issn><issn pub-type="epub">2949-2807</issn><publisher><publisher-name>ООО «Альфмед»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.33667/2078-5631-2021-33-30-34</article-id><article-id custom-type="elpub" pub-id-type="custom">medalphabet-2322</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>РЕВМАТОЛОГИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>RHEUMATOLOGY</subject></subj-group></article-categories><title-group><article-title>Осложнения и причины летальности у пациентов с первичными некротизирующими системными васкулитами</article-title><trans-title-group xml:lang="en"><trans-title>Complications and causes of mortality in patients with primary necrotizing systemic vasculitis</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>Мазуров</surname><given-names>В. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Mazurov</surname><given-names>V. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Мазуров Вадим Иванович, д.м.н., акад. РАН, зав. кафедрой</p><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Mazurov Vadim I., DM Sci (habil.), RAS academician, head of Dept</p><p>Saint Petersburg</p></bio><email xlink:type="simple">maz.nwgmu@yandex.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/0000-0002-7981-6349</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Беляева</surname><given-names>И. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Belyaeva</surname><given-names>I. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Беляева Ирина Борисовна, д.м.н., проф., врач-ревматолог; доцент кафедры</p><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Belyaeva Irina B., DM Sci (habil.), professor, rheumatologist; associate professorat Dept</p><p>Saint Petersburg</p></bio><email xlink:type="simple">belib@mail.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-7675-5683</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чудинов</surname><given-names>А. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Chudinov</surname><given-names>A. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Чудинов Антон Леонидович, к.м.н., зав. отделением</p><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Chudinov Anton L., PhD Med, head of Dept</p><p>Saint Petersburg</p></bio><email xlink:type="simple">anton-chudinov@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-9126-3639</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Инамова</surname><given-names>О. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Inamova</surname><given-names>O. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Инамова Оксана Владимировна, к.м.н., гл. врач</p><p>Санкт-Петербург</p></bio><bio xml:lang="en"><p>Inamova Oksana V., PhD Med, chief physician</p><p>Saint Petersburg</p></bio><email xlink:type="simple">ovig74@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБОУ ВО «Северо-Западный государственный медицинский университет имени И. И. Мечникова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>North-Western State Medical University n. a. I. I. Mechnikov</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>СПб ГБУЗ «Клиническая ревматологическая больница № 25»; ФГБОУ ВО «Северо-Западный государственный медицинский университет имени И. И. Мечникова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Clinical Rheumatology Hospital No. 25; North-Western State Medical University n. a. I. I. Mechnikov</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>СПб ГБУЗ «Клиническая ревматологическая больница № 25»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Clinical Rheumatology Hospital No. 25</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2021</year></pub-date><pub-date pub-type="epub"><day>13</day><month>12</month><year>2021</year></pub-date><volume>0</volume><issue>33</issue><issue-title>Ревматология в общей врачебной практике (2)</issue-title><fpage>30</fpage><lpage>34</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Мазуров В.И., Беляева И.Б., Чудинов А.Л., Инамова О.В., 2021</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="ru">Мазуров В.И., Беляева И.Б., Чудинов А.Л., Инамова О.В.</copyright-holder><copyright-holder xml:lang="en">Mazurov V.I., Belyaeva I.B., Chudinov A.L., Inamova O.V.</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://www.med-alphabet.com/jour/article/view/2322">https://www.med-alphabet.com/jour/article/view/2322</self-uri><abstract><p>В статье представлен анализ особенностей клинического течения, а также основных осложнений у пациентов c АНЦА- ассоциированными системными васкулитами (гранулематоз с полиангиитом, микроскопический полиангиит, эозинофильный гранулематоз с полиангиитом) и больных узелковым полиартериитом в течение первых трех лет заболевания. Оценена взаимосвязь осложнений с длительной глюкокортикостероидной терапией. Больные гранулематозом с полиангиитом имели наиболее неблагоприятное течение заболевания с высоким риском осложнений. Выявлена высокая частота развития ишемических инсультов и формирования хронической болезни почек у больных с микроскопическим полиангиитом. Проанализированы случаи осложнений и летальных исходов при первичных некротизирующих системных васкулитах.</p></abstract><trans-abstract xml:lang="en"><p>The article reviews the clinical symptoms, main complications in patients with ANCA-associated systemic vasculitis (granulomatous with polyangiitis, microscopic polyangiitis, eosinophilic granulomatous with polyangiitis) and polyarteritis nodosa during the first three years of the disease. We evaluated the relationship between complications and long-term glucocorticosteroid therapy. Patients with granulomatosis had the most unfavourable course of the disease with a high risk of complications. A high incidence of ischemic strokes and chronic kidney disease in patients with microscopic polyangiitis has been identified. We also analyzed cases of lethal outcomes in patients with primary systemic vasculitis.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>АНЦА-ассоциированные системные васкулиты</kwd><kwd>гранулематоз с полиангиитом</kwd><kwd>микроскопический полиангиит</kwd><kwd>эозинофильный гранулематоз с полиангиитом</kwd><kwd>узелковый полиартериит</kwd><kwd>осложнения</kwd></kwd-group><kwd-group xml:lang="en"><kwd>ANCA-associated systemic vasculitis</kwd><kwd>granulomatous with polyangiitis</kwd><kwd>microscopic polyangiitis</kwd><kwd>eosinophilic granulomatous with polyangiitis</kwd><kwd>polyarteritis nodosa</kwd><kwd>complications</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">Клиническая ревматология. 3-е издание, переработанное и дополненное. 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