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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">nid</journal-id><journal-title-group><journal-title xml:lang="ru">Нефрология и диализ</journal-title><trans-title-group xml:lang="en"><trans-title>Nephrology and Dialysis</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1680-4422</issn><issn pub-type="epub">2618-9801</issn><publisher><publisher-name>Российское диализное общество</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.28996/1680-4422-2017-4-466-477</article-id><article-id custom-type="elpub" pub-id-type="custom">nid-409</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 СТАТЬИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL ARTICLES</subject></subj-group></article-categories><title-group><article-title>Клинико-морфологическая характеристика и прогноз разных гистоморфологических вариантов быстропрогрессирующего гломерулонефрита, ассоциированного с АНЦА-васкулитом</article-title><trans-title-group xml:lang="en"><trans-title>Clinical and morphological characteristics and prognosis of different histomorphological variants of rapidly progressing glomerulonephritis associated with ANCA-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>Томилина</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Tomilina</surname><given-names>N. A.</given-names></name></name-alternatives><email xlink:type="simple">natomilina@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>Бирюкова</surname><given-names>Л. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Biryukova</surname><given-names>L. S.</given-names></name></name-alternatives><email xlink:type="simple">noemail@neicon.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Фролова</surname><given-names>Н. Ф.</given-names></name><name name-style="western" xml:lang="en"><surname>Frolova</surname><given-names>N. D.</given-names></name></name-alternatives><email xlink:type="simple">noemail@neicon.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Столяревич</surname><given-names>Е. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Stolyarevich</surname><given-names>E. S.</given-names></name></name-alternatives><email xlink:type="simple">noemail@neicon.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>Волгина</surname><given-names>Г. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Volgina</surname><given-names>G. V.</given-names></name></name-alternatives><email xlink:type="simple">noemail@neicon.ru</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Фролов</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Frolov</surname><given-names>A. V.</given-names></name></name-alternatives><email xlink:type="simple">noemail@neicon.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Тырин</surname><given-names>В. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Tyrin</surname><given-names>V. V.</given-names></name></name-alternatives><email xlink:type="simple">noemail@neicon.ru</email><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФБГУ "ФНЦ Трансплантологии и искусственных органов им. академика В.И. Шумакова" Министерства здравоохранения Российской Федерации; ФДПО ФГБУ ФГОУ "Московский государственный медико-стоматологический университет им. А.И. Евдокимова"; ГБУЗ "ГКБ №52 Департамента здравоохранения г. Москвы", Московский городской нефрологический центр</institution><country>Россия</country></aff><aff xml:lang="en"><institution>V.I. Shumakov Federal National Research Center of Transplantology and Artificial Organs; A.I. Evdokimov Moscow State University of Medicine and Dentistry; 20 Delegatskaya Str., bld.1, 127473 Moscow, Russian Federation; Moscow City Nephrology Center, Moscow City Hospital 52</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФДПО ФГБУ ФГОУ "Московский государственный медико-стоматологический университет им. А.И. Евдокимова"; ГБУЗ "ГКБ №52 Департамента здравоохранения г. Москвы", Московский городской нефрологический центр</institution><country>Россия</country></aff><aff xml:lang="en"><institution>A.I. Evdokimov Moscow State University of Medicine and Dentistry; 20 Delegatskaya Str., bld.1, 127473 Moscow, Russian Federation; Moscow City Nephrology Center, Moscow City Hospital 52</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>ГБУЗ "ГКБ №52 Департамента здравоохранения г. Москвы", Московский городской нефрологический центр</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Moscow City Nephrology Center, Moscow City Hospital 52</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>ФДПО ФГБУ ФГОУ "Московский государственный медико-стоматологический университет им. А.И. Евдокимова"</institution><country>Россия</country></aff><aff xml:lang="en"><institution>A.I. Evdokimov Moscow State University of Medicine and Dentistry; 20 Delegatskaya Str., bld.1, 127473 Moscow, Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2017</year></pub-date><pub-date pub-type="epub"><day>16</day><month>08</month><year>2024</year></pub-date><volume>19</volume><issue>4</issue><fpage>466</fpage><lpage>477</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Томилина Н.А., Бирюкова Л.С., Фролова Н.Ф., Столяревич Е.С., Волгина Г.В., Фролов А.В., Тырин В.В., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Томилина Н.А., Бирюкова Л.С., Фролова Н.Ф., Столяревич Е.С., Волгина Г.В., Фролов А.В., Тырин В.В.</copyright-holder><copyright-holder xml:lang="en">Tomilina N.A., Biryukova L.S., Frolova N.D., Stolyarevich E.S., Volgina G.V., Frolov A.V., Tyrin V.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://journal.nephro.ru/jour/article/view/409">https://journal.nephro.ru/jour/article/view/409</self-uri><abstract><p>Введение: васкулит, ассоциированный с антителами к цитоплазме нейтрофилов (ААВ), является одной из наиболее частых причин быстропрогрессирующего гломерулонефрита (БПГН). В условиях современной терапии его прогноз остается весьма серьезным, главным образом, в связи с токсичностью длительной иммуносупрессии. Поиски оптимальных предикторов исхода ААВ-БПГН имеют важное значение. Цель: анализ клинико-морфологических корреляций, ближайших и отдаленных результатов лечения БПГН при ААВ в зависимости от гистоморфологического характера заболевания. Материал и методы: ретроспективно проанализированы результаты лечения 115 больных с ААВ-БПГН в возрасте 56±14,3 лет (40% старше 60 лет) с уровнем креатинина сыворотки крови 570 [380; 830] мкмоль/л. По гистоморфологической картине БПГН больные разделены на 4 категории: с очаговым (22 больных), экстракапиллярным (34 больных), склерозирующим (30 больных), смешанным (29 больных) БПГН. Индукционная терапия состояла в комбинации пульсов метилпреднизолона и циклофосфана с преднизолоном внутрь. У 21 больного применен ритуксимаб. Для поддерживающей терапии применялись преднизолон и азатиоприн или микофенолаты. Результаты: к концу индукционной терапии улучшение достигнуто у 77% больных: в 100% при очаговом БПГН, в 80% - при смешанном, в 57% при экстракапиллярном и в 52% при склерозирующем БПГН. В целом в группе 5-летняя выживаемость больных составляла 67,7%, в возрасте старше 60 лет она снижалась до 38% против 80% в группе более молодых пациентов. 5-летняя почечная выживаемость равнялась 59% и зависела от гистоморфологической категории БПГН: при очаговом она составляла 89%, при смешанном 78%, при экстракапиллярном 56%, при склерозирующем 34% (p&lt;0,05). Заключение: полученные результаты демонстрируют тесную связь между ответом на терапию и гистоморфологическим вариантом БПГН. В меньшей степени это относится к ближайшим результатам, но отчетливо проявляется при оценке отдаленных результатов лечения.</p></abstract><trans-abstract xml:lang="en"><p>Background: anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is the most common cause of rapidly progressing glomerulonephritis (RPGN). Its prognosis has improved, however long-term patient morbidity and mortality still remain relatively high due to toxic effects of immunosuppressive treatment. Therefore, a search for optimal predictors of long-term outcome of this disease is of particular importance. Aim: to evaluate the short- and long-term outcomes of AAV-RPGN and their dependence the histopathologic classification. Materials and Methods: 115 biopsy-proven AAV-RPGN patients, aged 56±14.3 years (40% older than 60), were included in the retrospective study. The serum creatinine level by the time of presentation was 570 [380; 830] µmol/L. According to histopathology, the patients were divided into 4 groups: focal (22 pts), crescentic (34 pts), sclerotic (33 pts), and mixed (29 pts) RPGN. Induction therapy was carried out with methylprednisolone and cyclophosphamide IV pulses followed by oral prednisolone. Rituximab was used in 21 patients. Maintenance therapy was carried out with prednisolone and azathioprine or MMF. Results: improvement was observed in 77% of patients: 100% in group with focal, 80% in mixed, 57% in crescentic, and 52% in sclerotic RPGN. Five-years patient survival rate was 67.7%. It decreased to 38% in the group of patients older than 60 years versus 80% in the younger group. Five-years renal survival was 59% and was closely associated with the categories of glomerular lesions: 89% in group with focal RPGN, 78% in mixed RPGN, 56% in crescentic, and 34% in sclerotic RPGN (p&lt;0.05). Conclusion: our results show that histopathologic category of glomerular lesions in AAV-RPGN has predictive value for renal outcome.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>быстропрогрессирующий гломерулонефрит</kwd><kwd>АНЦА-ассоциированный васкулит</kwd><kwd>гистоморфологический вариант</kwd><kwd>ближайшие и отдаленные результаты лечения</kwd><kwd>anti-neutrophil cytoplasmic antibody-associated vasculitis</kwd><kwd>rapidly progressive glomerulonephritis</kwd><kwd>short-term and long-term outcomes</kwd><kwd>histopathologic category of glomerular lesions</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">Егорова Е.Т., Томилина Н.А., Бирюкова Л.С. и др. Быстропрогрессирующий гломерулонефрит при АНЦА-ассоциированных васкулитах: течение, эффективность лечения, прогноз. Нефрология и диализ. 2007. 9. 3: 228-239. E.T. Egorova, N.A. Tomilina, L.S. Birukova et al. 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