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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">nefr</journal-id><journal-title-group><journal-title xml:lang="ru">Нефрология</journal-title><trans-title-group xml:lang="en"><trans-title>Nephrology (Saint-Petersburg)</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1561-6274</issn><issn pub-type="epub">2541-9439</issn><publisher><publisher-name>Pavlov First Saint-Petersburg State Medical University</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.36485/1561-6274-2020-24-2-52-59</article-id><article-id custom-type="elpub" pub-id-type="custom">nefr-1800</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. КЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL ARTICLES. CLINICAL INVESTIGATIONS</subject></subj-group></article-categories><title-group><article-title>Опыт применения ритуксимаба при резистентном к терапии фокально-сегментарном гломерулосклерозе у взрослых: серия наблюдений и краткий обзор литературы</article-title><trans-title-group xml:lang="en"><trans-title>Experience of application of rituximab with resistant focal-segmental glomerulosclerosis in adults: a series of observations and a brief review of literature</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2128-8560</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чеботарева</surname><given-names>Н. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Chebotareva</surname><given-names>N. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Проф. Чеботарева Наталья Викторовна, д-р мед. наук.</p><p>Кафедра внутренних, профессиональных болезней и ревматологии Института клинической медицины им. Н.В.Склифосовского</p><p>119991, Москва, ул. Трубецкая, д. 8, стр. 2.Тел.: 8-905-543-42-50 </p><p> </p></bio><bio xml:lang="en"><p>Prof. Natalia V. Chebotareva, MD, PhD, DMedSci.</p><p>Department of Internal, Occupational Diseases and Rheumatology of the Institute of Clinical Medicine named after N.V. Sklifosovsky.</p><p>119991, Moscow, ul. Trubetskaya, d. 8, p. 2.tel. 8-905-543-42-50 </p><p> </p></bio><email xlink:type="simple">natasha_tcheb@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/0000-0002-8360-7734</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кучиева</surname><given-names>А. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Kuchieva</surname><given-names>A. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кучиева Агунда Мэлсовна - врач отделения «искусственная почка» Клиники нефрологии, внутренних и профессиональных болезней УКБ №3.</p><p>119991, Москва, ул. Трубецкая, д. 8, стр. 2.Тел.: 8-906-044-03-97 </p></bio><bio xml:lang="en"><p>Kuchieva Agunda Melsovna, MD - doctor of the artiﬁcial kidney department of the Clinic for Nephrology, Internal and Occupational Diseases, Clinical Hospital No. 3.</p><p>119991, Moscow, ul. Trubetskaya, d. 8, p. 2.tel. 8-906-044-03-97 </p><p> </p></bio><email xlink:type="simple">agunda_81@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/0000-0003-1739-677X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ли</surname><given-names>О. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Lee</surname><given-names>O. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Ли Ольга Александровна - врач отделения «искусственная почка» Клиники нефрологии, внутренних и профессиональных болезней УКБ №3.</p><p>119991, Москва, ул. Трубецкая, д. 8, стр. 2.Тел.: 8-925-829-16-06 </p></bio><bio xml:lang="en"><p>Li Olga Alexandrovna, MD - doctor of the artiﬁcial kidney department of the Clinic for Nephrology, Internal and Occupational Diseases, Clinical Hospital No. 3.</p><p>119991, Moscow, ul. Trubetskaya, d. 8, p. 2.tel. 8-925-829-16-06 </p></bio><email xlink:type="simple">shoa1978@bk.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>Рощупкина</surname><given-names>С. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Roshchupkina</surname><given-names>C. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Рощупкина Светлана Васильевна - заведующий отделением нефрологии Клиники нефрологии, внутренних и профессиональных болезней УКБ №3.</p><p>119991, Москва, ул. Трубецкая, д. 8, стр. 2.Тел.: 8-916-614-41-81 </p></bio><bio xml:lang="en"><p>Roshchupkina Svetlana Vasilievna, MD - Head of the Department of Nephrology, Clinic for Nephrology, Internal and Occupational Diseases, Clinical Hospital No. 3.</p><p>119991, Moscow, ul. Trubetskaya, d. 8, p. 2.tel. 8-916-614-41-81 </p></bio><email xlink:type="simple">roschupkina.sv@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/0000-0003-2694-4547</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Цао</surname><given-names>В.</given-names></name><name name-style="western" xml:lang="en"><surname>Cao</surname><given-names>V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Цао Веньцзин - аспирант кафедры внутренних, профессиональных болезней и ревматологии, Институт клинической медицины им. Н.В.Склифосовского.</p><p>119991, Москва, ул. Трубецкая, д. 8, стр. 2.Тел.: 8-8-916-903-72-38 </p></bio><bio xml:lang="en"><p>Cao Wenjing - Postgraduate student, MD.</p><p>Department of Internal, Occupational Diseases and Rheumatology of the Institute of Clinical Medicine named after N.V. Sklifosovsky.</p><p>119991, Moscow, ul. Trubetskaya, d. 8, p. 2.tel. 8-8-916-903-72-38 </p></bio><email xlink:type="simple">lrwj101031@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)</institution><country>Россия</country></aff><aff xml:lang="en"><institution>First Moscow State Medical University named after I.M. Sechenov (Sechenov University)</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2020</year></pub-date><pub-date pub-type="epub"><day>08</day><month>02</month><year>2020</year></pub-date><volume>24</volume><issue>2</issue><fpage>52</fpage><lpage>59</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Чеботарева Н.В., Кучиева А.М., Ли О.А., Рощупкина С.В., Цао В., 2020</copyright-statement><copyright-year>2020</copyright-year><copyright-holder xml:lang="ru">Чеботарева Н.В., Кучиева А.М., Ли О.А., Рощупкина С.В., Цао В.</copyright-holder><copyright-holder xml:lang="en">Chebotareva N.V., Kuchieva A.M., Lee O.A., Roshchupkina C.V., Cao 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.nephrolog.ru/jour/article/view/1800">https://journal.nephrolog.ru/jour/article/view/1800</self-uri><abstract><p>Стероид-резистентный фокально-сегментарный гломерулосклероз (ФСГС) с нефротическим синдромом (НС) является прогрессирующим заболеванием почек с быстрым развитием терминальной почечной недостаточности (тПН). В последние годы предпринимаются попытки лечения стероид-резистентного ФСГС анти-CD20-моноклональными антителами, ритуксимабом. В настоящее время опубликованы лишь единичные клинические наблюдения и серии, результаты которых противоречивы. ЦЕЛЬЮ нашего исследования было оценить эффективность применения ритуксимаба у больных с резистентным к терапии фокально-сегментарным гломерулосклерозом. ПАЦИЕНТЫ И МЕТОДЫ. В исследование вошли 6 пациентов с морфологически подтвержденным диагнозом ФСГС. Критериями включения были наличие НС, резистентного к терапии высокими дозами кортикостероидов и другим схемам лечения, включая циклоспорин А, такролимус, микофенолата мофетил, циклофосфамид, и исключение вторичной формы заболевания. Пациентам проведен один курс лечения ритуксимабом от 2 до 4 инфузий (суммарная доза составила 1,0–2,0 г). Лабораторные параметры (суточная протеинурия, альбумин и рСКФCKD-EPI) анализировали до назначения ритуксимаба, а также через 6 и 12 мес после завершения лечения. РЕЗУЛЬТАТЫ. В результате терапии у одного пациента достигнута частичная ремиссия ФСГС, стабильная функция почек сохранялась в течение 12 мес наблюдения. У 4 из 6 пациентов наблюдалось дальнейшее прогрессирование с развитием тПН в сроки от 1 до 3 лет, несмотря на уменьшение выраженности НС через 12 мес после введения ритуксимаба. У одного пациента сохранялся НС прежней выраженности в течение 6 мес после введения ритуксимаба, значимой положительной динамики со стороны протеинурии и белков сыворотки крови не отмечено. ЗАКЛЮЧЕНИЕ. Таким образом, мы не выявили значимого эффекта ритуксимаба у больных с резистентным к терапии ФСГС. Однако, учитывая эффективность применения препарата приблизительно у половины больных ФСГС по данным литературы, необходимы дальнейшие проспективные клинические исследования.</p></abstract><trans-abstract xml:lang="en"><p>Steroid-resistant focal-segmental glomerulosclerosis (FSGS) with nephrotic syndrome (NS) is a progressive kidney disease with the rapid development of end-stage renal failure (ESRD). In recent years, attempts have been made to treat steroidresistant FSHS with anti-CD20 monoclonal antibodies, rituximab. Currently, only a few clinical case reports and series with contradictory results have been published. THE AIM of our study was to evaluate the effect of rituximab in patients with resistant to the therapy focal segmental glomerulosclerosis. MATERIALS AND METHODS: The study included 6 patients with confirmed diagnosis of FSGS. The inclusion criteria were the presence of steroid-resistant NS or to the other treatment regimens, including cyclosporin A, tacrolimus, mycophenolate mofetil, cyclophosphamide, and exclusion criteria were the secondary form of the disease. Patients were treated with one course of rituximab – 2 to 4 infusions (the total dose was 1.0–2.0 g). Laboratory parameters (daily proteinuria, albumin and eGFR CKD-EPI) were analyzed before rituximab, as well as 6 and 12 months after treatment. RESULTS: A partial remission of FSGS was achieved in one patient, stable renal function was maintained for 12 months follow-up. In 4 out of 6 patients, progression of the disease was observed with the development of ESRD in the period from 1 to 3 years, despite a decrease in the severity of NS. In one patient, the NS of the same severity persisted for 6 months after the treatment; no positive dynamics in proteinuria or serum proteins were observed. CONCLUSION: Thus, we did not show a significant effect of rituximab in patients with steroid-resistant FSGS. However, according to the literature the effect of rituximab has been achieved in approximately half of steroid-resistant FSGS cases, therefore further prospective clinical trials are needed.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>фокально-сегментарный гломерулосклероз</kwd><kwd>нефротический синдром</kwd><kwd>ритуксимаб</kwd></kwd-group><kwd-group xml:lang="en"><kwd>focal segmental glomerulosclerosis</kwd><kwd>nephrotic syndrome</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">D'Agati V. The many masks of focal segmental glomerulosclerosis. Kidney Int 1994;46(4):1223–1241. doi https://doi.org/10.1038/ki.1994.388</mixed-citation><mixed-citation xml:lang="en">D'Agati V. The many masks of focal segmental glomerulosclerosis. 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