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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">10.47360/1995-4484-2022-450-454</article-id><article-id custom-type="elpub" pub-id-type="custom">rsp-3198</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>Ассоциации дигитальной ишемии и тяжести интерстициального заболевания легких у пациентов с системной склеродермией: данные 5-летнего наблюдения</article-title><trans-title-group xml:lang="en"><trans-title>Relationship between digital ulcers and severity of lung function test in systemic sclerosis over a five-year period</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2667-4284</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Овсянникова</surname><given-names>О. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Ovsyannikova</surname><given-names>O. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><p>115522, Moscow, Kashirskoye Highway, 34A</p></bio><email xlink:type="simple">sorry_84@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/0000-0003-3650-7658</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Конева</surname><given-names>О. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Koneva</surname><given-names>O. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><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/0000-0002-3248-6426</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ананьева</surname><given-names>Л. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Ananieva</surname><given-names>L. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><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/0000-0002-5012-0540</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гарзанова</surname><given-names>Л. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Garzanova</surname><given-names>L. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><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/0000-0002-1004-9647</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Старовойтова</surname><given-names>М. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Srarovoytova</surname><given-names>M. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><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/0000-0002-0283-9681</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Десинова</surname><given-names>О. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Desinova</surname><given-names>O. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><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/0000-0003-4688-9637</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шаяхметова</surname><given-names>Р. У.</given-names></name><name name-style="western" xml:lang="en"><surname>Shayakhmetova</surname><given-names>R. U.</given-names></name></name-alternatives><bio xml:lang="ru"><p>115522, Москва, Каширское шоссе, 34а</p></bio><bio xml:lang="en"><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"><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><pub-date pub-type="collection"><year>2022</year></pub-date><pub-date pub-type="epub"><day>06</day><month>09</month><year>2022</year></pub-date><volume>60</volume><issue>4</issue><fpage>450</fpage><lpage>454</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Овсянникова О.Б., Конева О.А., Ананьева Л.П., Гарзанова Л.А., Старовойтова М.Н., Десинова О.В., Шаяхметова Р.У., 2022</copyright-statement><copyright-year>2022</copyright-year><copyright-holder xml:lang="ru">Овсянникова О.Б., Конева О.А., Ананьева Л.П., Гарзанова Л.А., Старовойтова М.Н., Десинова О.В., Шаяхметова Р.У.</copyright-holder><copyright-holder xml:lang="en">Ovsyannikova O.B., Koneva O.A., Ananieva L.P., Garzanova L.A., Srarovoytova M.N., Desinova O.V., Shayakhmetova R.U.</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/3198">https://rsp.mediar-press.net/rsp/article/view/3198</self-uri><abstract><sec><title>Введение</title><p>Введение. Интерстициальное заболевание легких (ИЗЛ) у пациентов с системной склеродермией (ССД) является наряду с легочной гипертензией одной из ведущих причин смерти. Однако предикторы плохого прогноза заболевания у таких больных изучены пока недостаточно.</p><p>Цель исследования – оценить взаимосвязь между дигитальной ишемией (ДИ), течением интерстициального заболевания легких и динамикой показателей функциональных легочных тестов (ФЛТ) у пациентов с системной склеродермией за 5-летний период.</p></sec><sec><title>Методы</title><p>Методы. В исследование включено 77 пациентов с достоверным диагнозом ССД и ИЗЛ (возраст на момент включения составил в среднем 46±13,2 года; давность заболевания от первого не-Рейно-синдрома – 7,4±6,6 года; 69% пациентов с лимитированной формой ССД; 93% женщин). Средняя длительность наблюдения составила 58,9±11,3 месяца. На основании данных мультиспиральной компьютерной томографии (МСКТ) органов грудной клетки пациенты были разделены на три группы: в 1-ю группу вошли 16 больных с положительной динамикой; во 2-ю группу – 39 без динамики, в 3-ю группу – 22 с отрицательной динамикой по МСКТ. Всем пациентам были проведены функциональные легочные тесты (ФЛТ). Определялись форсированная жизненная емкость легких (ФЖЕЛ) и диффузионная способность легких (ДСЛ).</p></sec><sec><title>Результаты</title><p>Результаты. За период наблюдения дигитальная ишемия (ДИ; рубчики, язвочки и некрозы) выявлена у 27 (35%) пациентов с ССД и ИПЛ. Самое большое количество пациентов с ДИ было во 2-й и 3-й группах – 14 и 9 пациентов соответственно.</p><p>У всех пациентов без ДИ (n=54) ФЖЕЛ за 5 лет статистически значимо увеличилась: в среднем – с 88,5±19 до 96±23% (p &lt; 0,05); в 1-й группе – с 92±20,5 до 106±19% (p&lt;0,05); во 2-й группе – с 87±18 до 94±23,5% (p&gt;&lt;0,05). ФЖЕЛ у пациентов с ДИ существенно не менялась и составляла в среднем 88±14 и 86±16% соответственно (p&gt;0,05); в 3-й группе она имела тенденцию к снижению (с 83±12,5 до 74±13%), но эти различия не достигали статистической значимости. За 5-летний период наблюдения ДСЛ снизилась у всех пациентов независимо от наличия ДИ. Однако в 1-й группе это снижение было незначительным. Во 2-й группе у пациентов без ДИ (n=24) ДСЛ снизилась в среднем с 65±16 до 60±11% (p&lt;0,05), у пациентов с ДИ (n=14) – с 61±15 до 57±14% (p&gt;&lt;0,05); в группе 3 у пациентов без ДИ (n=13) – с 55±15 до 48±15% (p&gt;&lt;0,05), у пациентов с ДИ (n=9) – с 50±20 до 44,5±15% (p&gt;&lt;0,05). Заключение. У пациентов без ДИ за 5-летний период наблюдения ФЖЕЛ статистически значимо увеличилась. Самые низкие значения ФЖЕЛ и ДСЛ как на момент включения в исследование, так и в конце наблюдения были выявлены в группе с отрицательной динамикой ИЗЛ по данным МСКТ. Ключевые слова: системная склеродермия, интерстициальное заболевания легких, дигитальная ишемия&gt;˂ 0,05), у пациентов с ДИ (n=14) – с 61±15 до 57±14% (p&lt;0,05); в группе 3 у пациентов без ДИ (n=13) – с 55±15 до 48±15% (p&gt;&lt;0,05), у пациентов с ДИ (n=9) – с 50±20 до 44,5±15% (p&gt;&lt;0,05). Заключение. У пациентов без ДИ за 5-летний период наблюдения ФЖЕЛ статистически значимо увеличилась. Самые низкие значения ФЖЕЛ и ДСЛ как на момент включения в исследование, так и в конце наблюдения были выявлены в группе с отрицательной динамикой ИЗЛ по данным МСКТ. Ключевые слова: системная склеродермия, интерстициальное заболевания легких, дигитальная ишемия&gt;˂ 0,05); в группе 3 у пациентов без ДИ (n=13) – с 55±15 до 48±15% (p&lt;0,05), у пациентов с ДИ (n=9) – с 50±20 до 44,5±15% (p&gt;&lt;0,05). Заключение. У пациентов без ДИ за 5-летний период наблюдения ФЖЕЛ статистически значимо увеличилась. Самые низкие значения ФЖЕЛ и ДСЛ как на момент включения в исследование, так и в конце наблюдения были выявлены в группе с отрицательной динамикой ИЗЛ по данным МСКТ. Ключевые слова: системная склеродермия, интерстициальное заболевания легких, дигитальная ишемия&gt;˂ 0,05), у пациентов с ДИ (n=9) – с 50±20 до 44,5±15% (p&lt;0,05). Заключение. У пациентов без ДИ за 5-летний период наблюдения ФЖЕЛ статистически значимо увеличилась. Самые низкие значения ФЖЕЛ и ДСЛ как на момент включения в исследование, так и в конце наблюдения были выявлены в группе с отрицательной динамикой ИЗЛ по данным МСКТ. Ключевые слова: системная склеродермия, интерстициальное заболевания легких, дигитальная ишемия&gt;˂ 0,05).</p></sec><sec><title>Заключение</title><p>Заключение. У пациентов без ДИ за 5-летний период наблюдения ФЖЕЛ статистически значимо увеличилась. Самые низкие значения ФЖЕЛ и ДСЛ как на момент включения в исследование, так и в конце наблюдения были выявлены в группе с отрицательной динамикой ИЗЛ по данным МСКТ. </p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Background</title><p>Background. Systemic sclerosis-related interstitial lung disease (SSc-ILD) is the leading cause of death in SSc. Predictors of the outcomes of ILD in SSc are under investigation.</p><p>Objective – to assess association of the digital ulcers with dynamics of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) in patients with SSc-ILD.</p></sec><sec><title>Methods</title><p>Methods. It was a longitudinal study involving 77 pts with SSc-ILD (mean age was 46±13.2; 69% have limited subset of the disease; 93% were female). The mean duration of follow up was 58.9±11.3 months. At the end of the study a number of pts with digital ulcers (DUs) was 27 (35%). Additionally 77 pts with SSc-ILD were investigated with HRCT and were divided into 3 groups; The group 1 (16 pts) with improvement; group 2 (39 pts) without any changes and group 3 (22 pts) with worsening of fibrosis. PFT (measurement of forced vital capacity (FVC) and diffusion lung capacity (DLco)) were made.</p></sec><sec><title>Results</title><p>Results. 27 (35%) pts with SSc-ILD had DUs at the end of the study. The most pts with DUs was in groups 2 and 3 (14 and 9) accordingly at the end of the study. After 5 years of follow up FVC increased significantly in all pts without DUs (n=54) from 88.5±19 to 96±23% (p&lt;0.05); in group 1 – from 92±20.5 to 106±19% (p&gt;&lt;0.05); in group 2 – from 87±18 to 94±23.5% (p&gt;&lt;0.05) and only in group 3 FVC was stable (88±22 and 87±24.5%) (p&gt;0.05). The mean value of FVC in all pts with DUs didn’t change (88±14 and 86±16%; p&gt;0.05) with tendency to decreasing in group 3 (from 83±12.5 to 74±13%; p&gt;0.05). After 5 years of follow up DLco declined significantly in all pts with or without DUs, however in the 1st group decline of DLco wasn’t significant. The decreasing of DLCO was more prominent in group 3 than in group 2. Therefore, in group 2 in patient without DU (n=24) – from 65±16 to 60±11% (p&lt;0.05) and in patients with DU (n=14) DLCO changed from 61±15 to 57±14% (p&gt;&lt;0.05). In group 3 in patients without DU (n=13) DLCO decreased from 55±15 to 48±15% (p&gt;&lt;0.05) and in patients with DU (n=9) – from 50±20 to 44.5±15% (p&gt;&lt;0.05). Conclusions. In patients without DUs significant increasing of FVC during 5 years long follow up was observed. The worsening of fibrosis on HRCT in pts with DUs was associated with the lowest value of FVC and DLco at the entry and at the end of the study. Key words: systemic sclerosis, interstitial lung disease, digital ulcers&gt;˂ 0.05) and in patients with DU (n=14) DLCO changed from 61±15 to 57±14% (p 0.05). In group 3 in patients without DU (n=13) DLCO decreased from 55±15 to 48±15% (p˂ 0.05) and in patients with DU (n=9) – from 50±20 to 44.5±15% (p˂ 0.05).</p></sec><sec><title>Conclusions</title><p>Conclusions. In patients without DUs significant increasing of FVC during 5 years long follow up was observed. The worsening of fibrosis on HRCT in pts with DUs was associated with the lowest value of FVC and DLco at the entry and at the end of the study. </p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>системная склеродермия</kwd><kwd>интерстициальное заболевания легких</kwd><kwd>дигитальная ишемия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>systemic sclerosis</kwd><kwd>interstitial lung disease</kwd><kwd>digital ulcers</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">Гусева НГ. Системная склеродермия и псевдосклеродермические синдромы. 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