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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">microcirculation</journal-id><journal-title-group><journal-title xml:lang="ru">Регионарное кровообращение и микроциркуляция</journal-title><trans-title-group xml:lang="en"><trans-title>Regional blood circulation and microcirculation</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1682-6655</issn><issn pub-type="epub">2712-9756</issn><publisher><publisher-name>Academician I.P. Pavlov First St. Petersburg State Medical University</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.24884/1682-6655-2019-18-1-48-54</article-id><article-id custom-type="elpub" pub-id-type="custom">microcirculation-293</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>Выявление системной склеродермии у пациентов с легочной гипертензией</article-title><trans-title-group xml:lang="en"><trans-title>Detection of systemic sclerosis in patients with pulmonary hypertension</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>Кляус</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Klyaus</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кляус Наталия Андреевна – аспирант кафедры внутренних болезней</p><p>197341, Санкт-Петербург, ул. Аккуратова, д. 2</p></bio><bio xml:lang="en"><p>Klyaus Nataliya A. – MD, postgraduate student of the department of the internal diseases</p><p>197341, Saint-Petersburg, Akkuratova street, 2 </p></bio><email xlink:type="simple">klyausn@mail.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>Симакова</surname><given-names>М. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Simakova</surname><given-names>M. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Симакова Мария Александровна – кандидат медицинских наук, старший научный сотрудник научно-исследовательской лаборатории кардиомиопатий</p><p>197341, Санкт-Петербург, ул. Аккуратова, д. 2</p></bio><bio xml:lang="en"><p>Simakova Maria A. – MD, PhD, senior researcher of the department of research laboratory of cardiomyopathies</p><p>197341, Saint-Petersburg, Akkuratova street, 2 </p></bio><email xlink:type="simple">maria.simakova@gmail.com</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>Маслянский</surname><given-names>А. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Maslyanskiy</surname><given-names>A. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Маслянский Алексей Леонидович – кандидат медицинских наук, старший научный сотрудник научно-исследовательской лаборатории ревматологии</p><p>197341, Санкт-Петербург, ул. Аккуратова, д. 2</p></bio><bio xml:lang="en"><p>Maslyanskiy Aleksey L. – MD, PhD, senior researcher of the department of research laboratory of rheumatology</p><p>197341, Saint-Petersburg, Akkuratova street, 2 </p></bio><email xlink:type="simple">esc_4@mail.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>Моисеева</surname><given-names>О. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Moiseeva</surname><given-names>O. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Моисеева Ольга Михайловна – доктор медицинских наук, директор института сердца и сосудов</p><p>197341, Санкт-Петербург, ул. Аккуратова, д. 2</p></bio><bio xml:lang="en"><p>Moiseeva Olga M. – MD, DSc, director of the institute of heart and vessels</p><p>197341, Saint-Petersburg, Akkuratova street, 2 </p></bio><email xlink:type="simple">moiseeva.cardio@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное учреждение «Национальный медицинский исследовательский центр имени В. А. Алмазова» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Almazov National Medical Research Centre</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2019</year></pub-date><pub-date pub-type="epub"><day>02</day><month>05</month><year>2019</year></pub-date><volume>18</volume><issue>1</issue><fpage>48</fpage><lpage>54</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Кляус Н.А., Симакова М.А., Маслянский А.Л., Моисеева О.М., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Кляус Н.А., Симакова М.А., Маслянский А.Л., Моисеева О.М.</copyright-holder><copyright-holder xml:lang="en">Klyaus N.A., Simakova M.A., Maslyanskiy A.L., Moiseeva O.M.</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://www.microcirc.ru/jour/article/view/293">https://www.microcirc.ru/jour/article/view/293</self-uri><abstract><sec><title>Введение</title><p>Введение. Проблема своевременного выявления легочной артериальной гипертензии (ЛАГ) тесно связана с вопросами диагностики системной склеродермии (ССД). Это особенно важно при маломанифестных формах заболевания, затрудняющих верификацию диагноза до появления картины правожелудочковой сердечной недостаточности, и низкой информированности врачей общей практики об имеющихся диагностических возможностях.</p><p>Цель – выявить наиболее ранние клинические признаки ЛАГ, ассоциированной с ССД, для своевременной ее диагностики.</p></sec><sec><title>Материал и методы</title><p>Материал и методы. В исследование был включен 51 пациент с лимитированной формой ССД. Оценены степень поражения кожи, уровни N­терминального мозгового натрийуретического пропептида (NT­proBNP) и мочевой кислоты, тип свечения и титр антинуклеарного фактора. Всем пациентам проведены эхокардиографическое исследование, капилляроскопия ногтевого ложа, исследование функций внешнего дыхания. При выявлении симптомов ЛАГ для ее подтверждения выполнялась катетеризация правых камер сердца.</p></sec><sec><title>Результаты</title><p>Результаты. ЛАГ обнаружена у 19 из 51 пациента, включенного в исследование. Пациенты с ЛАГ оказались значительно старше больных без ЛАГ: (60±8) и (54±7) лет соответственно (р=0,02); между этими группами установлены различия по наличию признаков ремоделирования правых камер сердца (p&lt;0,05) в показателях, оцениваемых в тесте с 6­минутной ходьбой (p&lt;0,05), в оценке диффузионной способности легких (p&lt;0,001), в уровняхNT­proBNPи мочевой кислоты (p&lt;0,001), в плотности капилляров ногтевого ложа (р=0,009); в полуколичественной оценке альтераций капилляров ногтевого ложа (p=0,022). Среди пациентов с ЛАГ, ассоциированной с ССД, 7 пациентам диагноз ССД впервые выставлен после обращения к кардиологам и верификации генеза ЛАГ, тогда как остальные 12 пациентов ранее наблюдались ревматологами. Больные ЛАГ характеризовались более длительным периодом до постановки диагноза ССД (p=0,043).</p></sec><sec><title>Заключение</title><p>Заключение. В исследовании продемонстрирована взаимосвязь между тяжестью поражения капилляров ногтевого ложа и ЛАГ. Таким образом, капилляроскопия ногтевого ложа, наряду с модифицированным кожным счетом G. Rodnan, при лимитированной форме ССД может использоваться для оценки вероятности наличия ЛАГ. Четкое выполнение алгоритмов диагностики позволит своевременно верифицировать диагноз и будет способствовать улучшению качества оказания медицинской помощи.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. The problem of early detection of pulmonary arterial hypertension (PAH) is closely related to the diagnosis of systemic sclerosis (SSc). It is especially important in patients with slight manifestations of the disease, making it diffcult to verify the diagnosis before the clinic of right ventricular heart failure, and low knowledge of the general practitioners of the available diagnostic opportunities.</p><p>The aim of the work was to identify the earliest clinical signs of PAH associated with SSc for its early diagnosis.</p></sec><sec><title>Material and methods</title><p>Material and methods. The study included 51 patients with limited SSc. The investigators evaluated the extent of skin lesion, levels of the level of N­terminal brain natriureticpropeptide (NT­proBNP) and uric acid, the ﬂuorescent pattern and titer of antinuclear factor. All patients underwent an echocardiography, nailfold videocapillaroscopy, a comprehensive assessment of external respiratory functions. When there were symptoms of PAH, right heart catheterization was performed to confrm it.</p></sec><sec><title>Results</title><p>Results. PAH was detected in 19 of the 51 patients included in the study. Patients with PAH were signifcantly older than those without PAH: (60±8) and (54±7) years, respectively (p=0.02); there were also differences between these groups in the signs of right cardiac remodeling (p&lt;0.05), in the indices estimated in the six­minute walk test (p&lt;0.05), in the assessment of lung diffusing capacity (p&lt;0.001), in levels of NT­proBNP and uric acid (p&lt;0.001), in the density of the nailfold capillaries (p=0.009); in a semiquantitative assessment of nailfold capillary alterations (p=0.022). Among patients with PAH associated with SSc, seven patients were diagnosed with SSc for the frst time after referring to cardiologists and verifying the genesis of PAH, while other 12 patients were previously observed by rheumatologists. Differences in the duration of the period from the onset of symptoms to the diagnosis of SSc (p=0.043) were revealed between these groups.</p></sec><sec><title>Conclusion</title><p>Conclusion. The study shows the probability of the presence of SSc in patients with suspected idiopathic PAH, that determines the necessity of additional immunological and instrumental examinations in such patients.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>легочная гипертензия</kwd><kwd>системная склеродермия</kwd><kwd>антинуклеарный фактор</kwd><kwd>капилляроскопия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>pulmonary hypertension</kwd><kwd>systemic sclerosis</kwd><kwd>antinuclear factor</kwd><kwd>capillaroscopy</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">ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension, 2015. 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