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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">10.47360/1995-4484-2024-309-318</article-id><article-id custom-type="elpub" pub-id-type="custom">rsp-3579</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>ПЕДИАТРИЧЕСКАЯ РЕВМАТОЛОГИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>PEDIATRIC RHEUMATOLOGY</subject></subj-group></article-categories><title-group><article-title>Оценка индекса повреждения и его динамика на фоне терапии генно-инженерными биологическими препаратами у пациентов с несистемными вариантами ювенильного артрита</article-title><trans-title-group xml:lang="en"><trans-title>Assessment of the damage index and its dynamics in patients with non-systemic variants of juvenile arthritis during the treatment with biologics</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1935-8585</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Колхидова</surname><given-names>З. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kolkhidova</surname><given-names>Z. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Колхидова Зарина Анатольевна</p><p>115522, Москва, Каширское шоссе, 34а</p><p> </p></bio><bio xml:lang="en"><p>Zarina A. Kolkhidova</p><p>115522, Moscow, Kashirskoye Highway, 34A</p></bio><email xlink:type="simple">kolkhidovaz@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1842-0348</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Никишина</surname><given-names>И. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Nikishina</surname><given-names>I. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><p>Irina P. Nikishina</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/0000-0002-4285-0869</contrib-id><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><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><p>Svetlana I. Glukhova</p><p>115522, Moscow, Kashirskoye Highway, 34A</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru">ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»<country>Россия</country></aff><aff xml:lang="en">V.A. Nasonova Research Institute of Rheumatology<country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>27</day><month>06</month><year>2024</year></pub-date><volume>62</volume><issue>3</issue><fpage>309</fpage><lpage>318</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Колхидова З.А., Никишина И.П., Глухова С.И., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Колхидова З.А., Никишина И.П., Глухова С.И.</copyright-holder><copyright-holder xml:lang="en">Kolkhidova Z.A., Nikishina I.P., Glukhova S.I.</copyright-holder><license 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/3579">https://rsp.mediar-press.net/rsp/article/view/3579</self-uri><abstract><p>Течение ювенильного идиопатического артрита (ЮИА) характеризуется высоким риском развития деструктивного поражения суставов, внесуставными изменениями и, как следствие, быстропрогрессирующей инвалидизацией. Для оценки функциональной недостаточности и степени необратимых повреждений часто используются опросник CHAQ (Childhood Health Assessment Questionnaire) и рентгенография, но оба метода имеют ограничения. Для комплексной оценки исходов ЮИА в педиатрической ревматологии используется индекс повреждения JADI (Juvenile Arthritis Damage Index), включающий оценку суставного (JADI-A, JADI – Articular Damage) и внесуставного (JADI-E, JADI – Extra-Articular Damage) поражения. Использование индекса JADI может стать эффективным инструментом для прогнозирования и мониторинга эффективности терапии ЮИА в условиях реальной клинической практики.</p><p>Цель исследования – выявить корреляции индекса JADI с особенностями клинических проявлений у пациентов с несистемными вариантами ЮИА, не имевших опыта применения генно-инженерных биологических препаратов (ГИБП) или ингибиторов Янус-киназ, и оценить возможность прогнозирования ответа на ГИБП с помощью JADI.</p><sec><title>Материалы и методы</title><p>Материалы и методы. В открытое проспективное исследование включено 150 детей с несистемными вариантами ЮИА, не имевших опыта применения ГИБП в анамнезе. Средний возраст составил 12,2±4,6 года; 60% девочек. 112 пациентов обследованы в динамике через 6 или 12 месяцев от начала терапии ГИБП. Всем пациентам проводилась оценка клинико-анамнестических данных и суставного статуса и подсчет индексов JADI-A и JADI-E.</p></sec><sec><title>Результаты</title><p>Результаты. На момент первичного обследования до назначения ГИБП наличие фиксированных повреждений, согласно индексам JADI, установлено у 50% пациентов: по JADI-A«+» – у 43%, по JADI-E«+» – у 23%, одновременно по обеим категориям – у 15% (n=23) детей. Индекс повреждения JADI-A был представлен преимущественно сгибательными контрактурами коленных (39%), локтевых суставов (28%), ограничением движений в шейном отделе позвоночника (26%). Внесуставные повреждения чаще определялись наличием асептического некроза костей, подтвержденного визуализирующими методами (41%) и значительной разницей длины конечностей (35%). Суставные повреждения ассоциировались с позитивностью по антинуклеарному фактору, ревматоидному фактору, антителам к циклическому цитруллинированному пептиду, отсутствием энтезитов, повышением скорости оседания эритроцитов и уровня С-реактивного белка, высокой активностью, согласно счету JADAS71 (Juvenile Arthritis Disease Activity Score), наличием функциональных нарушений по CHAQ, глюкокортикоидной терапией в анамнезе и низкой приверженностью лечению. Характер дебюта в виде полиартрита с вовлечением мелких суставов кистей, склонностью к быстрому формированию контрактур также ассоциировался с положительным JADI-A. В результате проведенной терапии с использованием ГИБП или тофацитиниба, назначенных в условиях обычной клинической практики, у подавляющего большинства пациентов было достигнуто сохранение стабильного статуса без признаков повреждений либо значение индекса повреждения осталось неизменным. У 26 (23%) пациентов показатели JADI-A улучшились за счет полного разрешения контрактур или увеличения амплитуды движений суставов. В 10 (9%) случаях отмечалось прогрессирование повреждений вне установленной взаимосвязи с выбором конкретного препарата, в том числе у 6 (5%) детей необратимые изменения возникли несмотря на применение таргетной терапии.</p></sec><sec><title>Выводы</title><p>Выводы. Индекс JADI является полезным и доступным для повседневной клинической практики инструментом для клинической оценки суставных и внесуставных повреждений, отражающих прогноз ЮИА, и объективным показателем эффективности терапии.</p></sec><sec><title> </title><p> </p></sec><sec><title> </title><p> </p></sec></abstract><trans-abstract xml:lang="en"><p>Juvenile idiopathic arthritis (JIA) is characterized by a high risk of joint destruction, extra-articular changes, and rapidly progressive disability. The CHAQ (Childhood Health Assessment Questionnaire) questionnaire and X-ray are often used to assess functional impairment and the degree of irreversible damage, but both methods have limitations. The Juvenile Arthritis Damage Index (JADI) has been developed to comprehensively assess the biological prognosis of JIA, which includes articular (JADI-A) and extra-articular (JADI-E) damage evaluation. The use of the JADI may be an effective tool for predicting and monitoring the effectiveness of JIA therapy in real clinical practice conditions.</p><p>The aim – to identify correlations of the JADI index with features of clinical manifestations in patients with non-systemic variants of JIA who had no experience with biologic therapy (B) and to evaluate the impact of lesions defined through the JADI damage index value on predicting response to B.</p><sec><title>Methods</title><p>Methods. This open prospective study included 150 children with non-systemic JIA variants with no history of biologic therapy. The mean age was 12.2±4.6 years, 60% were girls. 112 patients were examined in dynamics after 6 or 12 months from the beginning of B therapy. Clinical and anamnestic evaluations, including detailed assessment of joint status and JADI-A and JADI-E scores, were performed on all patients.</p></sec><sec><title>Results</title><p>Results. The study found that 50% of patients had damage on the JADI score, with 43% having JADI-A+ and 23% having JADI-E+. Both articular and extra-articular damage were present in 15% (n=23) of children. Among those with JADI-A, flexion contractures of the knee joints were most frequently noted in 39%, elbow joints in 28%, and limitation of movement in the cervical spine in 26%. Extra-articular injuries were more frequently presented as avascular necrosis of bones, confirmed by imaging methods (41%), and a significant difference in limb length was observed in 35% of patients. The presence of articular changes was significantly associated with positivity for antinuclear antibody , rheumatoid factor, and anticyclic citrullinated peptide, as well as the absence of enthesitis, elevated erythrocyte sedimentation rate and С-reactive protein, high clinical activity, Juvenile Arthritis Disease Activity Score, CHAQ values, a history of glucocorticoid therapy, and low adherence to treatment. Polyarticular onset involving small hand joints and a tendency for rapid contracture formation were also associated with positive JADI-A values. The onset of polyarthritis with involvement of the small joints of the hands and a tendency to fast contracture formation were also associated with JADI-A positivity. As a result of therapy with B prescribed in routine clinical practice, the vast majority of patients achieved stable status without signs of damage or the value of the damage index remained unchanged. In 26 (23%) patients, JADI-A scores improved with complete resolution of contractures or increased amplitude of joint movement. In 10 (9%) cases there was progression of the lesions with no established correlation with the choice of a specific drug, including 6 (5%) children with irreversible changes despite the use of targeted therapy.</p></sec><sec><title>Conclusions</title><p>Conclusions. The JADI index is a useful and accessible tool for clinical assessment of articular and extra-articular lesions, reflecting the prognosis of JIA and an objective indicator of therapy efficacy.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>ювенильный артрит</kwd><kwd>индекс повреждения JADI</kwd><kwd>контрактуры суставов</kwd><kwd>функциональные нарушения</kwd><kwd>эффективность терапии</kwd><kwd>исходы ювенильного артрита</kwd><kwd>генно-инженерные биологические препараты</kwd><kwd>прогнозирование ответа на ГИБП</kwd></kwd-group><kwd-group xml:lang="en"><kwd>juvenile arthritis</kwd><kwd>JADI damage index</kwd><kwd>joint contractures</kwd><kwd>functional impairment</kwd><kwd>efficacy of therapy</kwd><kwd>outcomes of juvenile arthritis</kwd><kwd>biologics</kwd><kwd>predicting response to biologics</kwd></kwd-group><funding-group xml:lang="ru"><funding-statement>Исследование выполнено в рамках фундаментальной научной темы № 1021051302580-4 «Выявление клинических фенотипов и прогнозирование вариантов течения аутоиммунных и аутовоспалительных ревматических заболеваний детского возраста»</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Viola S, Felici E, Magni-Manzoni S, Pistorio A, Buoncompagni A, Ruperto N, et al. 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