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<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="research-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Russian Journal of Allergy</journal-id><journal-title-group><journal-title xml:lang="en">Russian Journal of Allergy</journal-title><trans-title-group xml:lang="ru"><trans-title>Российский Аллергологический Журнал</trans-title></trans-title-group></journal-title-group><issn publication-format="print">1810-8830</issn><issn publication-format="electronic">2686-682X</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">16521</article-id><article-id pub-id-type="doi">10.36691/RJA16521</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Original studies</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Оригинальные исследования</subject></subj-group><subj-group subj-group-type="article-type"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">New coronavirus infection SARS-CoV-2 in adult patients with inborn errors of immunity</article-title><trans-title-group xml:lang="ru"><trans-title>Течение новой коронавирусной инфекции SARS-CoV-2 у взрослых пациентов с врождёнными дефектами иммунитета</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5132-1267</contrib-id><contrib-id contrib-id-type="spin">7249-4423</contrib-id><name-alternatives><name xml:lang="en"><surname>Roppelt</surname><given-names>Anna A.</given-names></name><name xml:lang="ru"><surname>Роппельт</surname><given-names>Анна Артуровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Cand. Sci. (Med.)</p></bio><bio xml:lang="ru"><p>канд. мед. наук</p></bio><email>roppelt_anna@mail.ru</email><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6646-4233</contrib-id><name-alternatives><name xml:lang="en"><surname>Mаrkina</surname><given-names>Ulyana A.</given-names></name><name xml:lang="ru"><surname>Маркина</surname><given-names>Ульяна Алексеевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>itchermd@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6534-5902</contrib-id><contrib-id contrib-id-type="spin">5806-7260</contrib-id><name-alternatives><name xml:lang="en"><surname>Bobrikova</surname><given-names>Elena N.</given-names></name><name xml:lang="ru"><surname>Бобрикова</surname><given-names>Елена Николаевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>elena.bobrikova.69@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4949-9178</contrib-id><contrib-id contrib-id-type="spin">2884-5000</contrib-id><name-alternatives><name xml:lang="en"><surname>Кruglova</surname><given-names>Tatiana S.</given-names></name><name xml:lang="ru"><surname>Круглова</surname><given-names>Татьяна Сергеевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>surckova.t@yandex.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3794-4991</contrib-id><contrib-id contrib-id-type="spin">7721-1941</contrib-id><name-alternatives><name xml:lang="en"><surname>Мukhina</surname><given-names>Olga A.</given-names></name><name xml:lang="ru"><surname>Мухина</surname><given-names>Ольга Алексеевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>mukhina.o.a@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9545-4720</contrib-id><contrib-id contrib-id-type="spin">1857-8154</contrib-id><name-alternatives><name xml:lang="en"><surname>Lebedkina</surname><given-names>Marina S.</given-names></name><name xml:lang="ru"><surname>Лебедкина</surname><given-names>Марина Сергеевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>marina.ivanova0808@yandex.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7053-3900</contrib-id><contrib-id contrib-id-type="spin">2891-1650</contrib-id><name-alternatives><name xml:lang="en"><surname>Andrenova</surname><given-names>Gerelma V.</given-names></name><name xml:lang="ru"><surname>Андренова</surname><given-names>Гэрэлма Владимировна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>Andrenovagv@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6209-387X</contrib-id><contrib-id contrib-id-type="spin">5893-5394</contrib-id><name-alternatives><name xml:lang="en"><surname>Chernov</surname><given-names>Anton A.</given-names></name><name xml:lang="ru"><surname>Чернов</surname><given-names>Антон Александрович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>sbornaya1med@yandex.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3874-4721</contrib-id><contrib-id contrib-id-type="spin">4713-9943</contrib-id><name-alternatives><name xml:lang="en"><surname>Alekseeva</surname><given-names>Ekaterina I.</given-names></name><name xml:lang="ru"><surname>Алексеева</surname><given-names>Екатерина Иосифовна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Dr. Sci. (Med.), Professor, corresponding member of the Russian Academy of Sciences</p></bio><bio xml:lang="ru"><p>д-р мед. наук, профессор, чл.-корр. РАН</p></bio><email>alekatya@yandex.ru</email><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff4"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1930-5424</contrib-id><contrib-id contrib-id-type="spin">4122-5565</contrib-id><name-alternatives><name xml:lang="en"><surname>Karaulov</surname><given-names>Alexander V.</given-names></name><name xml:lang="ru"><surname>Караулов</surname><given-names>Александр Викторович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Dr. Sci. (Med.), Professor, academician of the Russian Academy of Sciences</p></bio><bio xml:lang="ru"><p>д-р мед. наук, профессор, академик РАН</p></bio><email>drkaraulov@mail.ru</email><xref ref-type="aff" rid="aff3"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6010-7975</contrib-id><contrib-id contrib-id-type="spin">3887-6250</contrib-id><name-alternatives><name xml:lang="en"><surname>Lysenko</surname><given-names>Mariana A.</given-names></name><name xml:lang="ru"><surname>Лысенко</surname><given-names>Марьяна Анатольевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Dr. Sci. (Med.), Professor</p></bio><bio xml:lang="ru"><p>д-р мед. наук, профессор</p></bio><email>gkb52@zdrav.mos.ru</email><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff5"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5083-6637</contrib-id><contrib-id contrib-id-type="spin">3023-4538</contrib-id><name-alternatives><name xml:lang="en"><surname>Fomina</surname><given-names>Daria S.</given-names></name><name xml:lang="ru"><surname>Фомина</surname><given-names>Дарья Сергеевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, Cand. Sci. (Med.), Assistant Professor</p></bio><bio xml:lang="ru"><p>канд. мед. наук, доцент</p></bio><email>daria_fomina@mail.ru</email><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff6"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Moscow City Hospital 52</institution></aff><aff><institution xml:lang="ru">Городская клиническая больница № 52</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology</institution></aff><aff><institution xml:lang="ru">Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии имени Дмитрия Рогачева</institution></aff></aff-alternatives><aff-alternatives id="aff3"><aff><institution xml:lang="en">The First Sechenov Moscow State Medical University (Sechenov University)</institution></aff><aff><institution xml:lang="ru">Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)</institution></aff></aff-alternatives><aff-alternatives id="aff4"><aff><institution xml:lang="en">National Medical Research Center for Children's Health</institution></aff><aff><institution xml:lang="ru">Национальный медицинский исследовательский центр здоровья детей</institution></aff></aff-alternatives><aff-alternatives id="aff5"><aff><institution xml:lang="en">The Russian National Research Medical University named after N.I. Pirogov</institution></aff><aff><institution xml:lang="ru">Российский национальный исследовательский медицинский университет имени Н.И. Пирогова</institution></aff></aff-alternatives><aff-alternatives id="aff6"><aff><institution xml:lang="en">Astana Medical University</institution></aff><aff><institution xml:lang="ru">Медицинский университет Астана</institution></aff></aff-alternatives><pub-date date-type="preprint" iso-8601-date="2024-05-14" publication-format="electronic"><day>14</day><month>05</month><year>2024</year></pub-date><pub-date date-type="pub" iso-8601-date="2024-06-16" publication-format="electronic"><day>16</day><month>06</month><year>2024</year></pub-date><volume>21</volume><issue>2</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>216</fpage><lpage>229</lpage><history><date date-type="received" iso-8601-date="2023-10-24"><day>24</day><month>10</month><year>2023</year></date><date date-type="accepted" iso-8601-date="2024-04-12"><day>12</day><month>04</month><year>2024</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2024, Pharmarus Print Media</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2024, Фармарус Принт Медиа</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="en">Pharmarus Print Media</copyright-holder><copyright-holder xml:lang="ru">Фармарус Принт Медиа</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/" start_date="2026-06-16"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by-nc-nd/4.0/</ali:license_ref></license></permissions><self-uri xlink:href="https://rusalljournal.ru/raj/article/view/16521">https://rusalljournal.ru/raj/article/view/16521</self-uri><abstract xml:lang="en"><p><italic>BACKGROUND: </italic>Diagnosis and treatment of COVID-19 in patients with primary immunodeficiency, or inborn errors of immunity, are often challenging.</p> <p><italic>AIM: </italic>Description of the COVID-19 course and therapy of adult patients with primary immunodeficiency treated in medical organizations of Moscow Healthcare Department.</p> <p><italic>MATERIALS AND METHODS: </italic>We analyzed a cohort of 68 patients over 18 years (median ― 35 years) with primary immunodeficiency; 91% of patients have primary immunodeficiency with predominantly antibody deficiencies. Altogether 90 cases of the new СOVID-19 were analyzed: in 68 cases infection occurred for the first time, in 22 cases it recurred. The duration of the disease ranged from 3 to 80 days. Duration of PCR-positivity ranged from 0 to 59 days, median 8 days.</p> <p><italic>RESULTS: </italic>Patients with Wuhan and Delta strains had more severe inflammatory signs according to C-reactive protein and lactate dehydrogenase, in patients with Wuhan lung involvement on CT-scam was larger. In demonstrated group of patients higher C-reactive protein correlated with larger lung involvement, longer duration of the disease and PCR-positivity, significant lymphopenia also correlated with higher C-reactive protein. To our data regularity of intravenous immunoglobulin therapy and IgG trough level didn’t correlate with infection severity and duration of the disease and virus-carriage.</p> <p>Indirectly, the change in the spectrum of medicine used in patients of the analyzed group coincided with the virus strain evolution. Anti-inflammatory therapy was mainly presented by dexamethasone and antagonists to interleukin 6 or its receptor (anti-IL-6): 55% and 73% for Wuhan, 63% and 50% for Delta, 17% and 39% for Omicron. Then, preference was gradually given to the target anti-cytokine medicine. Etiotropic antiviral therapy was more often used to treat infection caused by Wuhan and Delta strains ― 32% and 38%, respectively (in 17% for Omicron). With shifting toward immunotherapy by specific against COVID-19 immunoglobulins and monoclonal antibody to SARS-CoV-2: 5% and 9% for Wuhan, 0% and 75% for Delta, 48% and 83% for Omicron, respectively. Immune and etiotropic therapy was not carried out in Wuhan in 39%, in Delta in 43%, in Omicron in 41% of cases. The overall mortality rate from COVID-19 in the analyzed group was 3%.</p> <p><italic>CONCLUSION: </italic>Patients with primary immunodeficiency represent a vulnerable group to the SARS-CoV-2 virus with a high risk of not only severe, but also a protracted and undulating course of infection, what must be taken into account for the correct interpretation of the patient's condition and the timely administration of the appropriate therapy.</p></abstract><trans-abstract xml:lang="ru"><p>Обоснование. Диагностика и лечение COVID-19 у пациентов с первичными иммунодефицитами, или врождёнными дефектами иммунитета, зачастую представляют сложности.</p> <p>Цель исследования ― описание течения и терапевтических подходов COVID-19 у взрослых пациентов с первичными иммунодефицитами, получавших лечение в медицинских организациях Департамента здравоохранения г. Москвы.</p> <p>Материалы и методы. Проведён анализ когорты из 68 пациентов старше 18 (медиана 35) лет с первичными иммунодефицитами, из них 91% страдал первичными иммунодефицитами преимущественно с дефектом антителообразования. Всего проанализировано 90 случаев COVID-19, из них в 68 инфекция возникла впервые, в 22 ― повторно. Длительность заболевания варьировала от 3 до 80 дней, положительный тест методом полимеразной цепной реакции (ПЦР-позитивность) определялся в сроки от 0 до 59 (медиана 8) дней.</p> <p>Результаты. Пациенты со штаммами Wuhan и Delta имели более тяжёлые признаки воспалительной реакции по лабораторным исследованиям С-реактивного белка и лактатдегидрогеназы; у пациентов с Wuhan объём поражения лёгких, согласно компьютерной томограмме, также был больше. В представленной группе пациентов более высокие значения С-реактивного белка коррелировали с бÓльшим объёмом поражения лёгких, большей длительностью заболевания и ПЦР-позитивностью, выраженная лимфопения также коррелировала с более высоким значением С-реактивного белка. Согласно нашим данным, регулярность терапии внутривенным иммуноглобулином и концентрация предтрансфузионного IgG не коррелировали с тяжестью течения инфекции или длительностью заболевания и вирусоносительства. Косвенным образом изменение спектра применяемых лекарственных препаратов совпадало со сменой штамма вируса. Противовоспалительная терапия была преимущественно представлена дексаметазоном и антагонистами к интерлейкину 6 или его рецептору (анти-IL-6) (55 и 73% при Wuhan, 63 и 50% при Delta, 17 и 39% при Omicron), при этом предпочтение постепенно отдавалось таргетно действующим антицитокиновым препаратам. Этиотропная противовирусная терапия чаще применялась для лечения инфекции, вызванной штаммами Wuhan и Delta (32 и 38% соответственно; 17% при Omicron), со смещением в пользу иммунотерапии специфическими иммуноглобулинами человека против COVID-19 и моноклональными антителами к SARS-CoV-2 (5 и 9% при Wuhan, 0 и 75% при Delta, 48 и 83% при Omicron). Иммунная или этиотропная терапия не проводилась при Wuhan в 39%, при Delta в 43%, при Omicron в 41% случаев. Общая смертность от COVID-19 в анализируемой нами группе составила 3%.</p> <p>Заключение. Пациенты с первичными иммунодефицитами представляют уязвимую к вирусу SARS-СoV-2 группу с высоким риском не только тяжёлого, но и затяжного и волнообразного течения инфекции, что необходимо учитывать для правильной интерпретации состояния пациента и своевременного назначения целесообразной терапии.</p></trans-abstract><kwd-group xml:lang="en"><kwd>primary immunodeficiency</kwd><kwd>inborn errors of immunity</kwd><kwd>adults</kwd><kwd>new coronavirus infection SARS-CoV-2</kwd><kwd>treatment</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>первичный иммунодефицит</kwd><kwd>врождённые дефекты иммунитета</kwd><kwd>взрослые</kwd><kwd>новая коронавирусная инфекция SARS-CoV-2</kwd><kwd>лечение</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">Rahman S, Montero MT, Rowe K, et al. 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