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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">10.47360/1995-4484-2021-158-163</article-id><article-id custom-type="elpub" pub-id-type="custom">rsp-3018</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>Сравнительная характеристика клинико-лабораторных особенностей болезни Шёгрена с антицентромерными антителами и «классического» субтипа заболевания</article-title><trans-title-group xml:lang="en"><trans-title>Comparative characteristics of the clinical and laboratory features of the primary Sjogren’s syndrome associated with anticentromere antibodies and the “classic” subtype of the disease</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4188-3578</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чальцев</surname><given-names>Б. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Chaltsev</surname><given-names>B. D.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Чальцев Богдан Дмитриевич</p><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><p>Bogdan D. Chaltsev</p><p>115522, Moscow, Kashirskoye Highway, 34A</p></bio><email xlink:type="simple">bodya92@inbox.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/0000-0002-1425-8622</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Васильев</surname><given-names>В. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Vasiliev</surname><given-names>V. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>123056, Москва, Грузинский пер., 3</p></bio><bio xml:lang="en"><p>Vladimir I. Vasiliev</p><p>115522, Moscow, Kashirskoye Highway, 34A</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3389-7064</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Пальшина</surname><given-names>С. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Palshina</surname><given-names>S. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><p>Svetlana G. Palshina</p><p>115522, Moscow, Kashirskoye Highway, 34A</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/0000-0001-8099-2107</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Торгашина</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Torgashina</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><p>Anna V. Torgashina</p><p>115522, Moscow, Kashirskoye Highway, 34A</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/0000-0002-2191-9361</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сокол</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Sokol</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><p>Evgeniya V. Sokol</p><p>115522, Moscow, Kashirskoye Highway, 34A</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/0000-0003-2314-1466</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хван</surname><given-names>Ю. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Khvan</surname><given-names>Yu. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><p>Yulia I. Khvan</p><p>115522, Moscow, Kashirskoye Highway, 34A</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/0000-0002-6123-2679</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Родионова</surname><given-names>Е. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Rodionova</surname><given-names>E. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>119021, Москва, ул. Тимура Фрунзе, 11</p></bio><bio xml:lang="en"><p>Ekaterina B. Rodionova</p><p>119021, Moscow, Timura Frunze str., 11</p></bio><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4601-0904</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сафонова</surname><given-names>Т. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Safonova</surname><given-names>T. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>119021, Москва, ул. Россолимо, 11а</p></bio><bio xml:lang="en"><p>Tatiana N. Safonova</p><p>119021, Moscow, Rossolimo str., 11A</p></bio><xref ref-type="aff" rid="aff-4"/></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><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Клинико-диагностический центр МЕДСИ</institution><country>Россия</country></aff><aff xml:lang="en"><institution>“MEDSI”</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Лечебный Центр</institution><country>Россия</country></aff><aff xml:lang="en"><institution>“Lechebny center”</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>ФГБНУ «Научно-исследовательский институт глазных болезней»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Eye Diseases</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2021</year></pub-date><pub-date pub-type="epub"><day>11</day><month>05</month><year>2021</year></pub-date><volume>59</volume><issue>2</issue><fpage>158</fpage><lpage>163</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Чальцев Б.Д., Васильев В.И., Пальшина С.Г., Торгашина А.В., Сокол Е.В., Хван Ю.И., Родионова Е.Б., Сафонова Т.Н., 2021</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="ru">Чальцев Б.Д., Васильев В.И., Пальшина С.Г., Торгашина А.В., Сокол Е.В., Хван Ю.И., Родионова Е.Б., Сафонова Т.Н.</copyright-holder><copyright-holder xml:lang="en">Chaltsev B.D., Vasiliev V.I., Palshina S.G., Torgashina A.V., Sokol E.V., Khvan Y.I., Rodionova E.B., Safonova T.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/3018">https://rsp.mediar-press.net/rsp/article/view/3018</self-uri><abstract><p>Цель исследования – сравнить клинические и лабораторные проявления в двух группах пациентов с болезнью Шёгрена (БШ): с антицентромерными антителами (АЦА) и без них; сравнить частоту и клинико-лабораторные характеристики лимфом в этих двух группах.Материалы и методы. В лаборатории интенсивных методов терапии ревматических заболеваний ФГБНУ НИИР им. В.А. Насоновой находились под наблюдением 119 пациентов с АЦА-позитивной БШ (БШ-АЦА+). Диагноз БШ устанавливался на основании отечественных критериев 2001 г., ССД-критериев АCR/EULAR 2013 г.. Диагностика поражений печени осуществлялась на основании оценки общего и биохимического анализов крови, коагулограммы, определения антимитохондриальных антител (АМА), а также морфологического исследования биоптатов. Диагноз первичного билиарного холангита (ПБХ) устанавливался в соответствии с рекомендациями Американской ассоциации по изучению заболеваний печени, Российской гастроэнтерологической ассоциации и Российского общества по изучению печени. Лимфомы диагностировались по данным гистологического, иммуногистохимического и молекулярного исследований биоптатов пораженных органов, согласно классификации Всемирной организации здравоохранения. У 37 из 119 пациентов диагностировано сочетание БШ с системной склеродермией (ССД), и они были исключены из дальнейшего анализа. Проведено сравнение клинико-лабораторных особенностей в группе БШ-АЦА+ (n=82) и группе с АЦА-негативной БШ (БШ-АЦА–; n=64). Представлена оценка клинико-лабораторных характеристик лимфом в группах БШ-АЦА+ (n=14) и БШ-АЦА– (n=10).Результаты. В группе БШ-АЦА+ выявлен более поздний возраст дебюта заболевания; длительность болезни до выявления лимфомы не различалась. При БШ-АЦА+ обнаружены более низкая частота ревматоидного фактора (РФ), антител к Ro (анти-Ro) и La (анти-La), снижения содержания С3-компонента комплемента, гипергаммаглобулинемии, повышения концентрации IgG и C-реактивного белка, скорости оседания эритроцитов, лейкопении и анемии. 51,2% пациентов с БШ-АЦА+ были серонегативны как по анти-Ro и антиLa, так и по РФ. При БШ-АЦА+ обнаружена более высокая частота АМА и повышения концентрации IgM. Частота криоглобулинемии и моноклональной секреции не различалась. Частота рецидивирующих паротитов при БШ-АЦА+ была статистически значимо ниже, различий по частоте и выраженности других признаков поражения слюнных и слезных желез не было. ПБХ/эпителиит билиарных протоков в рамках БШ при БШ-АЦА+ выявлялся статистически значимо чаще. Поражение периферической нервной системы, легких, гипергаммаглобулинемическая пурпура, артралгии и аутоиммунный тиреоидит статистически значимо чаще выявлялись в группе БШ-АЦА–. В группе БШ-АЦА+ статистически значимо чаще встречался феномен Рейно, преимущественно с капилляроскопическими изменениями склеродермического типа. Разницы по частоте других признаков, характерных для ССД, не было. MALT-лимфомы диагностированы в исследуемых группах с одинаковой частотой. Пациенты с лимфомами при БШ-АЦА– характеризовались статистически значимо более выраженной лабораторной активностью. У всех пациентов с лимфомами в двух группах наблюдалось стойкое увеличение околоушных слюнных желез. Лимфомы одинаково часто развивались у пациентов с поздними стадиями поражения слюнных и слезных желез, системные проявления БШ в обеих группах встречались редко.Выводы. БШ-АЦА+ является самостоятельным субтипом БШ, имеющим ряд существенных клинических и лабораторных отличий от «классического» варианта заболевания (БШ-АЦА–). АЦА при БШ ассоциированы с низкой частотой анти-Ro, анти-La и РФ, а также с повышенным риском ПБХ и лимитированной формы ССД. MALT-лимфомы при БШ-АЦА+ и БШ-АЦА– развиваются с одинаковой частотой при прогрессировании железистых изменений, независимо от наличия или отсутствия системных проявлений.</p></abstract><trans-abstract xml:lang="en"><p>Objective: to compare clinical and laboratory manifestations in 2 groups of patients with primary Sjogren’s syndrome (pSS): with and without anticentromere antibodies (ACA); compare the incidence, clinical and laboratory characteristics of lymphomas in these two groups.Materials and methods. We examined 119 patients with ACA-positive pSS (pSS-ACA+). pSS was diagnosed based on Russian 2001 criteria, systemic sclerosis (SSc) – criteria ACR/EULAR 2013. To diagnose liver diseases, the level of transaminases, alkaline phosphatase and antimitochondrial antibodies (AMA) was determined, as well as liver biopsy. The diagnosis of primary biliary cholangitis (PBC) was established according to the recommendations of the American Association for the Study of Liver Diseases, the Russian Gastroenterological Association and the Russian Society for the Study of the Liver. Lymphomas were diagnosed according to histological, immunohistochemical and molecular studies of affected organs biopsies, according to the classification of the World Health Organization. A combination of pSS and SSc was diagnosed in 37 patients, and they were excluded from further analysis. We compared clinical and laboratory features in patients with pSS-ACA+ (n=82) and ACA-negative pSS (pSS-ACA–, n=64) and characterized lymphomas in the pSS-ACA+ (n = 14) and pSS-ACA– (n=10) groups.Results and discussion. In patients with pSS-ACA+, a later age of disease onset was revealed, the duration of the disease before lymphoma development did not differ. In patients with pSS-ACA+, we found a lower frequency of rheumatoid factor (RF), antibodies to Ro (anti-Ro) and La (anti-La), decreased C3-complement, hypergammaglobulinemia, increased IgG concentration, CRP, increased ESR, leukopenia and anemia. 51.2% of patients with pSS-ACA+ were seronegative for anti-Ro, anti-La and RF. Patients with pSS-ACA+ had a higher frequency of AMA and elevated IgM. The incidence of cryoglobulinemia and paraproteinemia did not differ. The frequency of recurrent parotitis in pSS-ACA+ was significantly lower, there were no differences in the frequency and severity of other signs of salivary and lacrimal gland damage. PBC and epitheliitis of the biliary ducts in patients with pSS-ACA+ were detected significantly more often. Damage to the peripheral nervous system, lungs, hypergammaglobulinemic purpura, arthralgia and autoimmune thyroiditis were significantly more often detected in the group of patients with pSS-ACA–. In the pSS-ACA+ group, Raynaud’s phenomenon was significantly more frequent, mainly with scleroderma-type capillaroscopic abnormalities. There was no difference in the frequency of other signs characteristic of SSc. MALT lymphomas were diagnosed in the study groups with the same frequency. Patients with lymphomas in the pSS-ACA+ group were characterized by significantly higher laboratory activity. All patients with lymphomas in both groups showed persistent parotid salivary gland enlargement. Lymphomas in both groups developed in patients with late stage salivary and lacrimal gland damage, systemic manifestations of pSS in both groups were rare.Conclusion. pSS-ACA+ is an independent subtype of pSS, which has a number of significant clinical and laboratory differences from the “classic” variant of the disease. ACA in pSS are associated with a low frequency of anti-Ro, anti-La, and RF, as well as an increased risk of PBC and limited SSc. MALT lymphomas in the pSS-ACA+ and pSS-ACA– groups developed with the same frequency and were associated with the progression of glandular damage, regardless of the presence of systemic manifestations.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>антицентромерные антитела</kwd><kwd>болезнь Шёгрена</kwd><kwd>системная склеродермия</kwd><kwd>первичный билиарный холангит</kwd><kwd>MALT-лимфома</kwd></kwd-group><kwd-group xml:lang="en"><kwd>anticentromere antibodies</kwd><kwd>primary Sjogren’s syndrome</kwd><kwd>systemic sclerosis</kwd><kwd>primary biliary cholangitis</kwd><kwd>MALT-lymphoma</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">Сафонова ТН, Васильев ВИ, Лихванцева ВГ. Синдром Шегрена: руководство для врачей. М.;2013:1-599.</mixed-citation><mixed-citation xml:lang="en">Safonova TN, Vasiliev VI, Likhvantseva VG. Sjögren’s syndrome: Guidelines for physicians. 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