<?xml version="1.0" encoding="UTF-8"?>
<!DOCTYPE root>
<article 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" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">The Clinician</journal-id><journal-title-group><journal-title xml:lang="en">The Clinician</journal-title><trans-title-group xml:lang="ru"><trans-title>Клиницист</trans-title></trans-title-group></journal-title-group><issn publication-format="print">1818-8338</issn><issn publication-format="electronic">2412-8775</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">342</article-id><article-id pub-id-type="doi">10.17650/1818-8338-2018-12-1-57-62</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>CASE REPORT</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>ОПИСАНИЕ СЛУЧАЯ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">STILL’S DISEASE IN A DULTS (CLINICAL CASES)</article-title><trans-title-group xml:lang="ru"><trans-title>БОЛЕЗНЬ СТИЛЛА У ВЗРОСЛЫХ (КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-9935-2126</contrib-id><name-alternatives><name xml:lang="en"><surname>Kanevskaya</surname><given-names>M. Z.</given-names></name><name xml:lang="ru"><surname>Каневская</surname><given-names>М. З.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>2/6 B. Pirogovskaya St., Moscow 119881</italic></p></bio><bio xml:lang="ru"><p><bold>Марина Зиновьевна Каневская </bold></p><p>Кафедра госпитальной терапии № 1</p><p><italic>119981 Москва, ул. Б. Пироговская, 2/6 </italic></p><p><bold/></p></bio><email>kanevskaya.m@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7938-1763</contrib-id><name-alternatives><name xml:lang="en"><surname>Kondratyeva</surname><given-names>T. B.</given-names></name><name xml:lang="ru"><surname>Кондратьева</surname><given-names>Т. Б.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>2/6 B. Pirogovskaya St., Moscow 119881</italic></p></bio><bio xml:lang="ru"><p>Кафедра госпитальной терапии № 1</p><p><italic>119981 Москва, ул. Б. Пироговская, 2/6 </italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">I. M. Sechenov First Moscow State Medical University, Ministry of Health of Russia</institution></aff><aff><institution xml:lang="ru">ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2018-01-15" publication-format="electronic"><day>15</day><month>01</month><year>2018</year></pub-date><volume>12</volume><issue>1</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>57</fpage><lpage>62</lpage><history><date date-type="received" iso-8601-date="2018-07-17"><day>17</day><month>07</month><year>2018</year></date><date date-type="accepted" iso-8601-date="2018-07-17"><day>17</day><month>07</month><year>2018</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2018, Kanevskaya M.Z., Kondratyeva T.B.</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2018, Каневская М.З., Кондратьева Т.Б.</copyright-statement><copyright-year>2018</copyright-year><copyright-holder xml:lang="en">Kanevskaya M.Z., Kondratyeva T.B.</copyright-holder><copyright-holder xml:lang="ru">Каневская М.З., Кондратьева Т.Б.</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://klinitsist.abvpress.ru/Klin/article/view/342">https://klinitsist.abvpress.ru/Klin/article/view/342</self-uri><abstract xml:lang="en"><p/><p><bold>Objective: </bold>to analyse and describe clinical cases of Still’s disease in adults and peculiarities of therapy in patient with a monocyclic course of the disease who had at the beginning of the observation a high systemic account (12) and in patient with a recurrent course of the disease with a positive result for 25 and 10 years of a dynamic observation.</p><p><bold>Materials and methods. </bold>Patient L., a female, 35 years old, was taken to the therapeutic department of the clinic with fever of 38–39 °С, chills and sweating, resistant not itching rash, lymphadenopathy, signs of myocarditis, loss of 20 kg of weight, exudative pleurisy, pronounced orthopnea with respiration rate of 40–48, tachycardia up to 130 beats per minute, myalgia, hepatosplenomegaly and abdominal pains. In past medical history for 2 months of the disease the patient was examined, and the sings of disease progression were present. The clinical constellation in patient L. allowed us to formulate a diagnosis of Still»s disease. Some investigations were conducted repeatedly to track the changes. The following therapy by Indomethacin in a daily dose of 150–200 mg/day for 1 year and for 6 months in combination with D-penicillamine in a dose of 450 mg/day induced remission. Till the end of 2016 the disease recurrence was not revealed. In another patient B., a male of 57 years old, the disease manifested with sub-febrile temperature, pain in a testicular area, thrombocytosis, and the symptoms increased in 2 weeks and included resistant febrile temperature, lymphadenopathy, changes of laboratory tests, signs of nephropathy and encephalitis. The patient was examined to exclude oncology.</p><p><bold>Results. </bold>Still’s disease was diagnosed and the following therapy was administrated: diclofenac and methotrexate for 4 years with episodes of disease recurrence in case of decreasing the doses of the medications, but in future the therapy managed to cancel, and there was no recurrence observed.</p><bold>Conclusion. </bold>These clinical cases have demonstrated peculiarities of course, diagnostic and management of patients with Still’s disease, the possibility of prescribing non-steroidal anti-inflammatory drugs and synthetic basic anti-inflammatory drugs for achievement of persistent disease remission.<italic> </italic></abstract><trans-abstract xml:lang="ru"><p><bold>Цель исследования </bold>– анализ и представление клинических случаев течения болезни Стилла взрослых (БСВ) и особенностей терапии у пациентки с моноциклическим течением заболевания, имевшей к началу наблюдения высокий системный счет (12), и у пациента с рецидивирующим течением заболевания и благоприятными исходами за 25 и 10 лет проспективного наблюдения.</p><p><bold>Материалы и методы. </bold>Пациентка Л., 35 лет, поступила в терапевтическое отделение клиники с лихорадкой 38–39 °С, ознобом и потливостью, стойкой не зудящей сыпью, лимфаденопатией, признаками миокардита, снижением массы тела на 20 кг, выпотным плевритом, выраженной одышкой (до 40–48 в минуту), ортопноэ, тахикардией до 130 уд/мин, миалгиями, гепатоспленомегалией, болями в животе. В анамнезе: за 2 мес болезни обследовалась и лечилась в различных стационарах без верификации диагноза с предполагаемым сепсисом и прогрессированием симптомов болезни. Совокупность проявлений заболевания позволили сформулировать диагноз БСВ при переводе в нашу клинику. Проводилась терапия индометацином по 150–200 мг/сут в течение 1 года и 6 мес в сочетании с Д-пеницилламином по 450 мг/сут с развитием ремиссии и моноциклическим течением за период наблюдения (до конца 2016 г.). У другого пациента Б., 57 лет, в дебюте заболевания отмечен субфебрилитет, боли в области яичка, тромбоцитоз и нарастание выраженности этих симптомов в течение 2 нед в виде стойкой фебрильной лихорадки, лимфаденопатии, изменения лабораторных показателей, признаков нефропатии и энцефалита. Проведены обследования по программе онкопоиска.</p><p><bold>Результаты. </bold>Установлен диагноз БСВ, терапию проводили диклофенаком и метотрексатом в течение 4 лет с рецидивами при снижении дозы препаратов; но в последующем терапию удалось отменить, и рецидивов заболевания в течение 4 лет наблюдения не было.</p><p><bold>Заключение. </bold>В данных клинических случаях представлены особенности течения, диагностики, ведения больных с БСВ и возможности назначения нестероидных противовоспалительных препаратов и синтетических базисных противовоспалительных препаратов в достижении стойкой ремиссии заболевания.</p></trans-abstract><kwd-group xml:lang="en"><kwd>prolonged fever</kwd><kwd>differential diagnosis</kwd><kwd>orphan diseases</kwd><kwd>Still’s disease in adults</kwd><kwd>therapy of Still’s disease in adults</kwd><kwd>prospective observation for 25 years associated with remission</kwd><kwd>prospective observation for 10 years with a recurrent course</kwd><kwd>monocyclic variant of clinical course of Still’s disease</kwd><kwd>recurrent variant of clinical course of Still’s disease</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>длительная лихорадка</kwd><kwd>дифференциальный диагноз</kwd><kwd>орфанные болезни</kwd><kwd>болезнь Стилла у взрослых</kwd><kwd>терапия болезни Стилла у взрослых</kwd><kwd>проспективное наблюдение в течение 25 лет на фоне ремиссии</kwd><kwd>проспективное наблюдение в течение 10 лет при рецидивирующем течении</kwd><kwd>моноциклический вариант течения болезни Стилла</kwd><kwd>рецидивирующий вариант течения болезни Стилла</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">Lebedev A. A. Rare diseases: problems and perspectives of solution. Zemskiy vrach = County Doctor 2011;6(10):5–9. (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Лебедев А. А. Редкие заболевания: проблемы и перспективы их решения. Земский врач 2011;6(10):5–9.</mixed-citation></citation-alternatives></ref><ref id="B2"><label>2.</label><mixed-citation>Bywaters E. G. Still’s disease in the adult. Ann Rheum Dis 1971;30(2):121–33.</mixed-citation></ref><ref id="B3"><label>3.</label><mixed-citation>Ohta A., Yamaguchi M., Kaneoka H. et al. Adult Still’s disease: review of 228 cases from literature. J Rheumatol 1987;14(6):1139–46.</mixed-citation></ref><ref id="B4"><label>4.</label><mixed-citation>Bannatyne G. A., Wohlmann A. S. Rheumatoid arthritis: its clinical history, etiology and pathology. Lancet 1896;(1):1120–5.</mixed-citation></ref><ref id="B5"><label>5.</label><mixed-citation>Magadur-Joly G., Billaud E., Barrier J. H. et al. Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 1995;54(7):587–90.</mixed-citation></ref><ref id="B6"><label>6.</label><mixed-citation>Wakai K., Ohta A., Tamakoshi A. et al. Estimated prevalence and incidence of adult Still’s disease; finding by a nationwide epidemiological survey in Japan. J Epidemiological 1997;7(4):221–5.</mixed-citation></ref><ref id="B7"><label>7.</label><mixed-citation>Ohta A., Yamaguchi M., Tsunematsu T. et al. Adult Still’s disease: a multicenter survey of Japanese patients. J Rheumatol 1990;17(8):1058–63.</mixed-citation></ref><ref id="B8"><label>8.</label><mixed-citation>Wouters J. M., Reekers P., van de Putte L. B. Adult-onset Still’s disease. Disease course and HLA associations. Arthr Rheumat 1986;29(3):415–8.</mixed-citation></ref><ref id="B9"><label>9.</label><mixed-citation>Wouters J. M., van de Putte L. B. Adultonset Still’s disease; clinical and laboratory features, treatment and progress of 45 cases. Quart J Med 1986;61(235):1055–65.</mixed-citation></ref><ref id="B10"><label>10.</label><mixed-citation>Pouchot J., Sampalis J. S., Beaudet F. et al. Adult Still’s Disease: Manifestations, Disease Course, and Outcome in 62 patients. Medicine (Baltimore) 1991;70(2):118–36.</mixed-citation></ref><ref id="B11"><label>11.</label><mixed-citation>Yamaguchi M., Ohta A., Tsunematsu T. et al. Preliminary criteria for classification of adul Still’s disease. J Rheumatol 1992;19(3):424–30.</mixed-citation></ref><ref id="B12"><label>12.</label><mixed-citation>Reginato A. J., Schumacher H. R., Baker D. G. et al. Adult onset Still’s disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum 1987;17(1):39–57.</mixed-citation></ref><ref id="B13"><label>13.</label><mixed-citation>Fautrel B., Zing E., Goldmart J. L. et al. Proposal for a new set of classification criteria for adult-onset Still disease. Medicine (Baltimore) 2002;81(3):194–200.</mixed-citation></ref><ref id="B14"><label>14.</label><mixed-citation>Franchini S., Dagna L., Salvo F. et al. Adult onset Still’s disease: clinical presentation in a large cohort of Italian patients. Clin Exp Rheumatol 2010;28(1):41–8. PMID: 20346237.</mixed-citation></ref><ref id="B15"><label>15.</label><mixed-citation>Lee S. W., Рark Y. B., Song J. S., Lee S. K. The mid-range of the adjusted level of ferritin can predict the chronic course in patients with adult onset Still’s disease. J Rheumatol 2009;36(1):156–62.</mixed-citation></ref><ref id="B16"><label>16.</label><mixed-citation>Ruscitti P., Cipriani P., Masedu F. et al. Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers. BMC Medicine 2016;14(1):194. DOI: 10.1186/s12916‑016‑0738‑8.</mixed-citation></ref><ref id="B17"><label>17.</label><mixed-citation>Cush J. J. Adult-onset Still’s disease. Bull Rheum Dis 2000;49(6):1–4.</mixed-citation></ref><ref id="B18"><label>18.</label><citation-alternatives><mixed-citation xml:lang="en">Nemcinov E. N., Kanevskaya M. Z., Yankin V.V. Adultonset Still’s disease: diagnostic criteria, variants of progression, clinical observations. In: Current problems of internal medicine and teaching. Moscow: MMA imeni I. M. Sechenova, 2002: 214–221. (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Немчинов Е. Н., Каневская М. З., Янкин В. В. Болезнь Стилла взрослых: критерии диагноза, варианты течения, клинические наблюдения. В кн.: Актуальные вопросы внутренней медицины и педагогики. М.: ММА им. И. М. Сеченова, 2002.</mixed-citation></citation-alternatives></ref><ref id="B19"><label>19.</label><mixed-citation>Kong X. D., Zhang W., Zhao Y., Zhang F. Clinical features in adult-onset Still’s disease: a study of 104 cases. Clin Rheumatol 2010;29(9):105–9. DOI: 10.1007/s10067‑010‑1516‑1.</mixed-citation></ref><ref id="B20"><label>20.</label><mixed-citation>Guilpain P., Le Quelles A. About the complexity of adult onset Still’s disease and advances still required for its management. BMC Med 2017;15(1):5. DOI: 10.1186/s12916‑016‑0769‑1.</mixed-citation></ref><ref id="B21"><label>21.</label><mixed-citation>Masson C., Le Loët X., Liote F. et al. Adult Still’s disease: part I. Manifestations and complications in sixty-five cases in France. Rev Rhum Engl Ed 1995;62(11):748–57.</mixed-citation></ref><ref id="B22"><label>22.</label><mixed-citation>Evensen K. J., Nossent H. C. Epidemiology and outcome of adult Still’s disease in northern Norway. Scand J Rheumatol 2006;35(1):48–51.</mixed-citation></ref><ref id="B23"><label>23.</label><mixed-citation>Tristano A. G. Macrophage activation syndrome: a frequent in patients with adult Still’s disease. Med Sci Monit 2008;14(3):RA27–36.</mixed-citation></ref><ref id="B24"><label>24.</label><mixed-citation>Masyama A., Kobayashi H., Kobayach Y. et al. A case of adult-onset Still’s disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema. Mod Rheumatol 2013;23(2):379–85.</mixed-citation></ref><ref id="B25"><label>25.</label><mixed-citation>Calabro J. J., Cruess R. L., Levinson J. E. et al. Juvenile rheumatoid arthritis. Patient Care 1977;(11):70–116.</mixed-citation></ref><ref id="B26"><label>26.</label><mixed-citation>Bujak J. S., Aptekar R. G., Decker J. L., Wolff S. M. Juvenile rheumatoid arthritis presenting in the adult as fever of unknown origin. Medicine(Baltimore) 1973; 52(5):431–44.</mixed-citation></ref><ref id="B27"><label>27.</label><mixed-citation>Shojania K., Chalmers A., Rangno K. Cyclosporin A in treatment of adult Still’s disease. J Rheumatol 1995;22(7):1391–2.</mixed-citation></ref><ref id="B28"><label>28.</label><mixed-citation>Cefle A. Leflunomide and azathioprine combination in refractory adult Still’s disease. Ann Pharmacother 2005;39(4):764–7.</mixed-citation></ref><ref id="B29"><label>29.</label><mixed-citation>Jamilloux Y., Gerfaud-Valentin M., Henry T., Seve P. Treatment of adult-onset Still’s disease: a review. Ther Clin Risk Manag 2014;11:33–43. DOI: 10.2147/TCRM.S64951.</mixed-citation></ref><ref id="B30"><label>30.</label><mixed-citation>Elkon K. B., Hughes G. R., Bywaters E. G. et al. Adult onset Still’s disease: Twentyyear follow-up and further studies of patients with active disease. Arthr Rheum 1982;25(6):647–54.</mixed-citation></ref><ref id="B31"><label>31.</label><citation-alternatives><mixed-citation xml:lang="en">Muravjev Yu. V. Adult-onset Still’s disease. Russian clinical guidelines. Rheumatology. Ed.: acad. E. L. Nasonov. Moscow: GEOTARMedia, 2017. 464 р. (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Муравьев Ю. В. Болезнь Стилла у взрослых. Российские клинические рекомендации. Ревматология. Под ред. акад. Е. Л. Насонова. M.: ГЭОТАР-Медиа, 2017. 464 с.</mixed-citation></citation-alternatives></ref><ref id="B32"><label>32.</label><mixed-citation>Hochino T., Ohta A., Yang D. et al. Elevated serum interleukin 6, interferongamma, and tumor necrosis factor-alpha levels in patients with adult Still’s disease. J Rheumatol 1998;25(2):396–8.</mixed-citation></ref><ref id="B33"><label>33.</label><mixed-citation>Kotter I., Wacker A., Koch S. et al. Anakinra in patients with treatmentresistant adult-onset Still’s disease: four case report with serial cytokine measurements and a review of the literature. Semin Arthr Rheum 2007;37(3):189–97.</mixed-citation></ref><ref id="B34"><label>34.</label><mixed-citation>Ogata A., Kitano M., Yamanaka J. et al. Interleukin 18 and hepatocyte grown factor in fulminant hepatic failure of adult onset Still’s disease. J Rheumatol 2003;30(5):1093–6.</mixed-citation></ref><ref id="B35"><label>35.</label><mixed-citation>Kontzias A., Efthimiou P. Adult-onset Still’s disease pathogenesis, clinical manifestations and therapeutic advances. Drugs 2008;68(3):319–37.</mixed-citation></ref><ref id="B36"><label>36.</label><citation-alternatives><mixed-citation xml:lang="en">Muravjev Y. V., Nasonov E. V. Adult-onset Still’s disease. Nauchno-prakticheskaya revmatologiya = Scientific and Practical Rheumatology 2010;2:58–65 (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Муравьев Ю. В., Насонов Е. В. Болезнь Стилла, развившаяся у взрослых. Научнопрактическая ревматология 2011;(2):58–65.</mixed-citation></citation-alternatives></ref></ref-list></back></article>
