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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">10.14412/1995-4484-2014-147-158</article-id><article-id custom-type="elpub" pub-id-type="custom">rsp-1918</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>Российский опыт применения моноклональных антител к В-лимфоцитам (ритуксимаб) при системных васкулитах, ассоциированных с антинейтрофильными цитоплазматическими антителами (предварительные результаты российского регистра НОРМА)</article-title><trans-title-group xml:lang="en"><trans-title>RUSSIAN EXPERIENCE WITH USING MONOCLONAL ANTIBODIES TO B-LYMPHOCYTES (RITUXIMAB) IN SYSTEMIC VASCULITIDES ASSOCIATED WITH NEUTROPHIL CYTOPLASMIC ANTIBODIES (PRELIMINARY RESULTS OF THE RUSSIAN REGISTER NORMA)</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>Бекетова</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Beketova</surname><given-names>T. V.</given-names></name></name-alternatives><email xlink:type="simple">tvbek@rambler.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>Александрова</surname><given-names>Е. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Aleksandrova</surname><given-names>E. N.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Новоселова</surname><given-names>Т. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Novoselova</surname><given-names>T. M.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сажина</surname><given-names>Е. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Sazhina</surname><given-names>E. G.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Николаева</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Nikolaeva</surname><given-names>E. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Смирнов</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Smirnov</surname><given-names>A. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сороцкая</surname><given-names>В. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Sorotskaya</surname><given-names>V. N.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Земерова</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Zemerova</surname><given-names>E. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Нам</surname><given-names>И. Ф.</given-names></name><name name-style="western" xml:lang="en"><surname>Nam</surname><given-names>I. F.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Никитина</surname><given-names>Н. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Nikitina</surname><given-names>N. M.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Архангельская</surname><given-names>Г. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Arkhangelskaya</surname><given-names>G. S.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Багаутдинова</surname><given-names>З. Р.</given-names></name><name name-style="western" xml:lang="en"><surname>Bagautdinova</surname><given-names>Z. R.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-6"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Дашков</surname><given-names>И. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Dashkov</surname><given-names>I. N.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-7"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Черных</surname><given-names>С. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Chernykh</surname><given-names>S. Yu.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-8"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Жирнова</surname><given-names>О. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Zhirnova</surname><given-names>O. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-9"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Лушпаева</surname><given-names>Ю. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Lushpaeva</surname><given-names>Yu. A.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-10"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Маснева</surname><given-names>Л. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Masneva</surname><given-names>L. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-11"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Афанасьева</surname><given-names>И. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Afanasieva</surname><given-names>I. P.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-12"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Арсеньев</surname><given-names>А. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Arseniev</surname><given-names>A. E.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-12"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кондратенко</surname><given-names>И. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Kondratenko</surname><given-names>I. V.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-13"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Башкова</surname><given-names>И. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Bashkova</surname><given-names>I. B.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-14"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Глухова</surname><given-names>С. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Glukhova</surname><given-names>S. I.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><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><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ «Научно-исследовательский институт ревматологии им. В.А.Насоновой» РАМН, Москва, Россия</institution><country>Россия</country></aff><aff xml:lang="en"><institution>V.A. Nasonova Research Institute of Rheumatology of the Russian Academy of Medical Sciences, Moscow, Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Медицинский институт ФГБОУ ВПО «Тульский государственный университет», Тула, Россия</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Medical Institute, Tula State University, Tula, Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>БУ ХМАО-Югры «Окружная клиническая больница», Ханты-Мансийск, Россия</institution><country>Россия</country></aff><aff xml:lang="en"><institution>County Clinical Hospital, Khanty-Mansiysk, Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>ГБОУ ВПО «Саратовский государственный медицинский университет им. В.И. Разумовского» Минздрава России, Саратов</institution><country>Россия</country></aff><aff xml:lang="en"><institution>V.I. Razumovsky State Medical University, Ministry of Health of Russia, Saratov</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>ГБУ Самарской области «Самарская городская больница № 4», Самара</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Samara City Hospital Four, Samara</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-6"><aff xml:lang="ru"><institution>БУЗ Удмуртской Республики «Республиканский клинико-диагностический центр Министерства здравоохранения Удмуртской Республики», Ижевск</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Republican Clinical and Diagnostic Center, Ministry of Health of the Udmurt Republic, Izhevsk</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-7"><aff xml:lang="ru"><institution>ГАУЗ «Брянская областная больница № 1», Брянск</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Bryansk Regional Hospital One, Bryansk</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-8"><aff xml:lang="ru"><institution>ОГАУЗ «Иркутская городская клиническая больница № 1», Иркутск</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Irkutsk City Clinical Hospital One, Irkutsk</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-9"><aff xml:lang="ru"><institution>ГБУЗ Тверской области «Областная клиническая больница», Тверь</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Tver Regional Clinical Hospital, Tver</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-10"><aff xml:lang="ru"><institution>Тюменский областной ревматологический  центр ГБУЗ ТО «Областная клиническая больница №1», Тюмень</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Tyumen Regional Rheumatology Center, Regional Clinical Hospital One, Tumen</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-11"><aff xml:lang="ru"><institution>ОГБУЗ «Белгородская областная клиническая больница Святителя Иоасафа», Белгород</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Saint Ioasaf Belgorod Regional Clinical Hospital, Belgorod</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-12"><aff xml:lang="ru"><institution>ОБУЗ «Ивановская областная клиническая больница», Иваново</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Ivanovo Regional Clinical Hospital, Ivanovo</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-13"><aff xml:lang="ru"><institution>ФГБУ «Российская детская клиническая больница» Минздрава России, Москва</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Russian Children’s Clinical Hospital, Ministry of Health of Russia, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-14"><aff xml:lang="ru"><institution>ФГБОУ ВПО «Чувашский государственный университет им. И.Н. Ульянова» , Чебоксары</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.N. Ulyanov Chuvash State University, Cheboksary</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2014</year></pub-date><pub-date pub-type="epub"><day>17</day><month>06</month><year>2014</year></pub-date><volume>52</volume><issue>2</issue><issue-title>НАУЧНО-ПРАКТИЧЕСКАЯ РЕВМАТОЛОГИЯ 52 No 2-2014</issue-title><fpage>147</fpage><lpage>158</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Бекетова Т.В., Александрова Е.Н., Новоселова Т.М., Сажина Е.Г., Николаева Е.В., Смирнов А.В., Сороцкая В.Н., Земерова Е.В., Нам И.Ф., Никитина Н.М., Архангельская Г.С., Багаутдинова З.Р., Дашков И.Н., Черных С.Ю., Жирнова О.В., Лушпаева Ю.А., Маснева Л.В., Афанасьева И.П., Арсеньев А.Е., Кондратенко И.В., Башкова И.Б., Глухова С.И., Насонов Е.Л., 2014</copyright-statement><copyright-year>2014</copyright-year><copyright-holder xml:lang="ru">Бекетова Т.В., Александрова Е.Н., Новоселова Т.М., Сажина Е.Г., Николаева Е.В., Смирнов А.В., Сороцкая В.Н., Земерова Е.В., Нам И.Ф., Никитина Н.М., Архангельская Г.С., Багаутдинова З.Р., Дашков И.Н., Черных С.Ю., Жирнова О.В., Лушпаева Ю.А., Маснева Л.В., Афанасьева И.П., Арсеньев А.Е., Кондратенко И.В., Башкова И.Б., Глухова С.И., Насонов Е.Л.</copyright-holder><copyright-holder xml:lang="en">Beketova T.V., Aleksandrova E.N., Novoselova T.M., Sazhina E.G., Nikolaeva E.V., Smirnov A.V., Sorotskaya V.N., Zemerova E.V., Nam I.F., Nikitina N.M., Arkhangelskaya G.S., Bagautdinova Z.R., Dashkov I.N., Chernykh S.Y., Zhirnova O.V., Lushpaeva Y.A., Masneva L.V., Afanasieva I.P., Arseniev A.E., Kondratenko I.V., Bashkova I.B., Glukhova S.I., Nasonov E.L.</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/1918">https://rsp.mediar-press.net/rsp/article/view/1918</self-uri><abstract><p>В 2013 г. в России официально зарегистрированы показания для применения моноклональных антител к В-лимфо- цитам (ритуксимаб, РТМ) при системных васкулитах, ассоциированных с антинейтрофильными цитоплазматиче- скими антителами (АНЦА-СВ). Данное сообщение представляет предварительные результаты Российского регист- ра применения РТМ при аутоиммунных заболеваниях (НОРМА), включившего 50 больных АНЦА-СВ, получавших лечение в 14 городах Российской Федерации. У 25 из 50 (50%) пациентов проводили повторные курсы РТМ. В условиях реальной отечественной клинической практики РТМ проявил высокую эффективность у больных АНЦА-СВ с хорошим профилем безопасности лечения. Среди 25 пациентов, наблюдавшихся более 12 мес, ремис- сия была достигнута в 92% случаев, в 8% наблюдалось снижение активности АНЦА-СВ. Эффективность РТМ воз- растала при использовании повторных курсов, при этом отмечено, что положительные результаты могут быть полу- чены при назначении повторных курсов РТМ в редуцированных дозах (500–1000 мг). Назначение повторных кур- сов в первую очередь потребовалось больным гранулематозом с полиангиитом с поражением легких. Требуется осторожность при сочетании лечения РТМ с цитостатиками (прежде всего с циклофосфаном) в связи с опасностью вторичного иммунодефицитного состояния и инфекционных нежелательных реакций (НР), которые были наиболее частыми серьезными НР (12%) у больных АНЦА-СВ. </p></abstract><trans-abstract xml:lang="en"><p>In 2013, Russia registered officially the indications for the use of monoclonal antibodies to B-lymphocytes (rituximab, RTM) in systemic vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA-SV). This communication presents the preliminary results of the Russian register of the RTM application in autoimmune diseases (NORMA) that has included 50 patients with ANCA-SV treated in 14 cities of the Russian Federation. Twenty-five of 50 (50%) patients received repeated courses of RTM. RTM has demonstrated a high efficacy and a good profile of treatment safety in patients with ANCA-SV in real-life national clinical practice. Among 25 patients who had been followed up for over 12 months, the remission was achieved in 92% of cases, a decrease in the ANCA-SV activity was observed in 8%. The efficacy of RTM increased when performing repeated courses, while it has been noted that the positive results can be obtained by prescribing a repeated course of RTM at a reduced dose (500–1000 mg). Prescription of the repeated courses was primarily required in patients with granulomatosis and polyangiitis affecting the lungs. Care should be taken when combining RTM treatment with cytostatics (primarily with cyclophosphamide) because of the risk of secondary immunodeficiency and infectious adverse events (AE), which have been the most frequent serious AE (12%) in patients with ANCA-SV. </p></trans-abstract><kwd-group xml:lang="ru"><kwd>системный васкулит</kwd><kwd>ассоциированный с антинейтрофильными цитоплазматическими антитела- ми</kwd><kwd>гранулематоз с полиангиитом Вегенера</kwd><kwd>микроскопический полиангиит</kwd><kwd>эозинофильный гранулематоз с поли- ангиитом Черджа–Строс</kwd><kwd>биологическая анти-В-клеточная терапия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>systemic vasculitis associated with neutrophil cytoplasmic antibodies</kwd><kwd>Wegener's granulomatosis with polyangiitis</kwd><kwd>microscopic polyangiitis</kwd><kwd>eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrom)</kwd><kwd>biological anti-B cell therapy</kwd><kwd>rituximab</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">Jones R, Cohen Tervaert J, Hauser T, et al. 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