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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">ssmu</journal-id><journal-title-group><journal-title xml:lang="ru">Бюллетень сибирской медицины</journal-title><trans-title-group xml:lang="en"><trans-title>Bulletin of Siberian Medicine</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1682-0363</issn><issn pub-type="epub">1819-3684</issn><publisher><publisher-name>Siberian State Medical University, the Ministry of Healthcare of the Russian Federation</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.20538/1682-0363-2018-2-167-174</article-id><article-id custom-type="elpub" pub-id-type="custom">ssmu-1214</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>ОБЗОРЫ И ЛЕКЦИИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEW AND LECTURES</subject></subj-group></article-categories><title-group><article-title>Патогенетические основы таргетной терапии рецидивирующего полихондрита</article-title><trans-title-group xml:lang="en"><trans-title>Pathogenetic mechanisms of biological agents in managing of relapsing polychondritis</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2220-7745</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Халева</surname><given-names>Е. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Khaleva</surname><given-names>E. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>ассистент, кафедра педиатрии им. профессора И.М. Воронцова,</p><p>194100, г. Санкт-Петербург, ул. Литовская, 2</p></bio><bio xml:lang="en"><p>Assistant, Department of Paediatrics,</p><p>2, Litovskaya Str., Saint-Petersburg, 194353</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-7571-5460</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Новик</surname><given-names>Г. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Novik</surname><given-names>G. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д-р мед. наук, профессор, зав. кафедрой педиатрии им. профессора И.М. Воронцова,</p><p>194100, г. Санкт-Петербург, ул. Литовская, 2</p></bio><bio xml:lang="en"><p>DM, Professor, Head of the Department of Paediatrics,</p><p>2, Litovskaya Str., Saint-Petersburg, 194353</p></bio><email xlink:type="simple">ga_novik@mail.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/0000-0003-2159-2722</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Рохлина</surname><given-names>Ф. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Rokhlina</surname><given-names>F. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>ассистент, кафедра педиатрии им. профессора И.М. Воронцова, 194100, г. Санкт-Петербург, ул. Литовская, 2;</p><p>врач-ревматолог, консультативно-диагностический центр, 196603, г. Санкт-Петербург, Пушкин, ул. Парковая, 64–68</p></bio><bio xml:lang="en"><p>Assistant, Department of Paediatrics, 2, Litovskaya Str., Saint-Petersburg, 194353;</p><p>Rheumatologist, 64-68, Parkovaya Str., Pushkin, Saint-Petersburg, 196603</p></bio><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Санкт-Петербургский государственный педиатрический медицинский университет (СПбГПМУ)</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Saint-Petersburg State Pediatric Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Санкт-Петербургский государственный педиатрический медицинский университет (СПбГПМУ);&#13;
Научно-исследовательский детский ортопедический институт (НИДОИ) имени Г.И. Турнера</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Saint-Petersburg State Pediatric Medical University&#13;
Research Сhildren’s Orthopedic Institute named after G.I. Turner</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2018</year></pub-date><pub-date pub-type="epub"><day>20</day><month>07</month><year>2018</year></pub-date><volume>17</volume><issue>2</issue><fpage>167</fpage><lpage>174</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Халева Е.Г., Новик Г.А., Рохлина Ф.В., 2018</copyright-statement><copyright-year>2018</copyright-year><copyright-holder xml:lang="ru">Халева Е.Г., Новик Г.А., Рохлина Ф.В.</copyright-holder><copyright-holder xml:lang="en">Khaleva E.G., Novik G.A., Rokhlina F.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://bulletin.ssmu.ru/jour/article/view/1214">https://bulletin.ssmu.ru/jour/article/view/1214</self-uri><abstract><p>Рецидивирующий полихондрит (РП) – редкое аутоиммунное заболевание, характеризующееся рецидивирующим воспалением хрящевой и других, богатых протеогликаном тканей организма. Сопутствующие заболевания, в частности миелодисплазия или cистемные аутоиммунные заболевания, могут быть обнаружены у одной трети пациентов с РП. В отличие от взрослых у детей РП реже ассоциирован с другими аутоиммунными заболеваниями. Диагноз РП устанавливается с использованием критериев Mc Adam (1976) или Damiani (1979).</p><p>Основой патогенеза РП является аутоиммунная реакция, которая первоначально направлена против хряща и далее распространяется на нехрящевые ткани. Одним из звеньев патогенеза РП является механическая травма хряща, приводящая к высвобождению провоспалительных цитокинов (фактор некроза опухоли-α, интерферон-γ, IL-8 и макрофагальный воспалительный белок 1) и локальному воспалению с последующим образованием аутоантител у пациента с генетической предрасположенностью. В лечении РП используют стероиды, нестероидные противовоспалительные препараты, колхицин и, если они неэффективны, то назначают иммунодепрессанты. Наиболее эффективными антицитокиновыми препаратами, используемыми в лечении РП, являются ингибиторы фактора некроза опухоли-α, антагонисты рецептора IL-1, ингибитор ко-стимулирующего пути активации Т-лимфоцитов, моноклональные антитела против рецептора IL-6. Учитывая сложность ведения таких пациентов, целью данного обзора было осветить последние данные о патогенетических основах антицитокиновой терапии РП. </p></abstract><trans-abstract xml:lang="en"><p>Relapsing polychondritis (RPC) is an autoimmune disease characterized by the inflammation of cartilaginous tissues and other proteoglycan rich tissues. A concomitant disease, particularly myelodysplasia or systemic autoimmune disease can be detected in one-third of the patients with RPC. Unlike adults in children, RPC is less often associated with other autoimmune diseases. The diagnosis of RPC is established using the criteria of Mc Adam (1976) or Damiani (1979). The basis of the pathogenesis of RPC is an autoimmune reaction, which is initially directed against cartilage and then spreads to non-cartilaginous tissues. One of the elements in the pathogenesis of RPC is the mechanical trauma of cartilage, resulting in the release of pro-inflammatory cytokines (tumor necrosis factor alpha, interferon-γ, interleukin-8, and macrophage inflammatory protein 1) and local inflammation followed by the formation of autoantibodies in a patient with a genetic predisposition. In the treatment of RPC, steroids, non-steroidal anti-inflammatory drugs, colchicine are used and, if they are ineffective, immunosuppressants are prescribed. The most effective anti-cytokine drugs used in the treatment of RPC are tumor necrosis factor-alpha (TNF-α) inhibitors, IL-1 receptor antagonists, an inhibitor of the costimulatory pathway of T-lymphocyte activation, monoclonal antibodies against the IL-6 receptor. Given the fact that management of these patients is very complex, the aim of the study is to review available data on pathogenetic mechanisms of biological agents in managing of relapsing polychondritis.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>рецидивирующий полихондрит</kwd><kwd>клиника</kwd><kwd>этиопатогенез</kwd><kwd>биологическая терапия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>relapsing polychondritis</kwd><kwd>clinics</kwd><kwd>etiopathogenesis</kwd><kwd>biological agents</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">Letko E., Zafirakis P., Baltatzis S. et al. Relapsing polychondritis: A clinical review. Seminars in Arthritis and Rheumatism. 2002; 31 (6): 384–395. DOI:10.1053/ sarh.2002.32586.</mixed-citation><mixed-citation xml:lang="en">Letko E., Zafirakis P., Baltatzis S. et al. Relapsing polychondritis: A clinical review. 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