mrjСовременная ревматологияModern Rheumatology Journal1996-70122310-158XIMA-PRESS, LLC10.14412/1996-7012-2021-3-27-34mrj-1144Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL INVESTIGATIONSАутоиммунные поражения печени у пациентов с болезнью Шёгрена, ассоциированной с антицентромерными антителамиAutoimmune liver damage in patients with primary Sjogren's syndrome associated with anticentromeric antibodieshttps://orcid.org/0000-0003-4188-3578ЧальцевБ. Д.ChalcevB. D.

Богдан Дмитриевич Чальцев

Россия, 115522, Москва, Каширское шоссе, 34А

Bogdan Dmitrievitch Chalcev

34A, Kashirskoe Shosse, Moscow 115522, Russia

bodya92@inbox.ruhttps://orcid.org/0000-0003-4350-1166ВасильевВ. И.VasilievV. I.

Россия, 123056, Москва, Грузинский переулок, 3А

3A, Gruzinskiy pereulok, Moscow 123056, Russia

https://orcid.org/0000-0003-3898-9769ПальшинаС. Г.PalshinaS. G.

Россия, 107140, Москва, Нижняя Красносельская ул., 4

4, Nizhniaya Krasnoselskaya, Moscow 107140, Russia

https://orcid.org/0000-0001-8099-2107ТоргашинаА. В.TorgashinaA. V.

Россия, 115522, Москва, Каширское шоссе, 34А

34A, Kashirskoe Shosse, Moscow 115522, Russia

https://orcid.org/0000-0002-2191-9361СоколЕ. В.SokolE. V.

Россия, 115522, Москва, Каширское шоссе, 34А

34A, Kashirskoe Shosse, Moscow 115522, Russia

https://orcid.org/0000-0003-2314-1466ХванЮ. И.KhvanJ. I.

Россия, 115522, Москва, Каширское шоссе, 34А

34A, Kashirskoe Shosse, Moscow 115522, Russia

https://orcid.org/0000-0002-6123-2679РодионоваЕ. Б.RodionovaE. B.

Россия, 119021, Москва, ул. Тимура Фрунзе, 15/1

15/1, Timura Frunze str., Moscow 119021, Russia

https://orcid.org/0000-0002-4601-0904СафоноваТ. Н.SafonovaT. N.

Россия, 119021, Москва, ул. Россолимо, 11А, Б

11А, Б, Rossolimo str., Moscow 119021, Russia

НекрасоваТ. П.NekrasovaT. P.

Россия, 119021, Москва, ул. Трубецкая, 8, стр. 2

8, Trubetskaya Str., Build 2, Moscow 119991, Russia

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian FederationАО «Группа компаний МЕДСИ», Москва; 3ООО «Ревмоцентр»РоссияJSC Group of companies MEDSIRussian FederationООО «Ревмоцентр»Россия«Rheumocenter» LLCRussian FederationООО «Лечебный центр»Россия«Lechebnyy Tsenter» LLCRussian FederationФГБНУ «Научно-исследовательский институт глазных болезней»РоссияResearch Institute of eye diseasesRussian FederationФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский университет)РоссияI.M. Sechenov First Moscow State Medical University of thr Ministry of Health of the Russian Federation (Sechenov University)Russian Federation2021230620211532734Copyright © Чальцев Б.Д., Васильев В.И., Пальшина С.Г., Торгашина А.В., Сокол Е.В., Хван Ю.И., Родионова Е.Б., Сафонова Т.Н., Некрасова Т.П., 20212021Чальцев Б.Д., Васильев В.И., Пальшина С.Г., Торгашина А.В., Сокол Е.В., Хван Ю.И., Родионова Е.Б., Сафонова Т.Н., Некрасова Т.П.Chalcev B.D., Vasiliev V.I., Palshina S.G., Torgashina A.V., Sokol E.V., Khvan J.I., Rodionova E.B., Safonova T.N., Nekrasova T.P.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://mrj.ima-press.net/mrj/article/view/1144

Цель настоящего исследования – определить частоту, спектр и тяжесть течения поражений печени у позитивных по антицентромерным антителам (АЦА) пациентов с болезнью Шёгрена (БШ).

Пациенты и методы

Пациенты и методы. У 37 (31%) из 119 включенных в исследование АЦА-позитивных пациентов с БШ при обследовании выявлены признаки поражения печени, 3 из них были исключены из исследования (у 2 имелась желчнокаменная болезнь, у 1 – вирусный гепатит В). Признаки аутоиммунного поражения печени обнаружены у 34 (28,6%) больных, большинство из которых были серопозитивны по антимитохондриальным антителам (АМА). Диагноз первичного билиарного холангита (ПБХ) и аутоиммунного гепатита (АИГ) устанавливался согласно рекомендациям Американской ассоциации по изучению заболеваний печени, Российской гастроэнтерологической ассоциации и Российского общества по изучению печени. Причина холестаза у 5 (14,7%) пациентов осталась неуточненной.

Результаты и обсуждение

Результаты и обсуждение. АМА обнаружены у 73,5% пациентов, повышение уровня IgM – у 57,6%. Клинически поражение печени в большинстве случаев характеризовалось бессимптомным медленно прогрессирующим течением, как правило, без выраженного нарастания симптомов в динамике. Признаки цирроза выявлены у 14,7% пациентов. На основании клинико-лабораторно-морфологических проявлений ПБХ диагностирован у 21 больного, у 4 из которых также имелся перекрестный синдром с АИГ. АМА-негативный ПБХ установлен у 3 пациентов и изолированный АИГ – у 1. В большинстве случаев определялась I гистологическая стадия ПБХ. При динамическом наблюдении, медиана которого составила 7 лет (мин. 2 года, макс. 15 лет), у 7 пациентов с I стадией ПБХ и у 7 АМА-позитивных больных без функциональных нарушений печени в течение всего периода наблюдения клинического и лабораторно-инструментального прогрессирования поражения печени не отмечено. В связи с этим сделано предположение, что у данных пациентов имеется эпителиит билиарных протоков как проявление железистых поражений при БШ, а не истинный ПБХ.

Заключение

Заключение. Аутоиммунные поражения печени выявляются у 28,6% АЦА-позитивных пациентов с БШ, у большинства из них с одинаковой частотой (41,2%) развиваются эпителиит билиарных протоков в рамках БШ либо сочетание БШ с ПБХ, реже диагностируется перекрестный синдром ПБХ/АИГ. ПБХ/эпителиит билиарных протоков в рамках БШ у АЦА-позитивных пациентов в большинстве случаев характеризуется медленно прогрессирующим бессимптомным течением и редко приводит к развитию цирроза печени.

Objective

Objective: to determine the frequency, spectrum and severity of liver affection in anti-centromere antibodies (ACA) positive patients with primary Sjogren's syndrome (pSS).

Patients and methods

Patients and methods. 119 ACA-positive patients with pSS were included in the study, 37 (31%) of them had signs of liver damage, 3 of these patients were excluded from the study (2 had cholelithiasis, 1 had viral hepatitis B). Signs of autoimmune liver damage were found in 34 (28.6%) patients, most of them were seropositive for antimitochondrial antibodies (AMA). The diagnosis of primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) was established according to the recommendations of the American Association for the Study of Liver Diseases, the Russian Gastroenterological Association and the Russian Society for the Study of the Liver. In 5 (14.7%) patients the cause of cholestasis remained unspecified.

Results and discussion

Results and discussion. AMA were found in 73.5% of patients, elevated serum IgM levels – in 57.6%. Clinically liver damage in most cases was characterized by an asymptomatic, slowly progressive course without a dramatic increase of symptoms over time. Liver cirrhosis was found in 14.7% of patients. According to clinical, laboratory and morphological manifestations, PBC was diagnosed in 21 patients, 4 of them also had a cross syndrome with AIH. AMA-negative PBC was found in 3 patients and isolated AIH – in 1. In most cases, histological stage I of PBC was detected. During follow-up, median of 7 years (range from 2 to 15 years), in 7 patients with stage I PBC and in 7 AMA-positive patients without functional liver disorders no clinical, laboratory or instrumental progression of liver damage was noted. In this regard, it was suggested that these patients have epitheliitis of the biliary ducts as manifestation of glandular affection in pSS, and not true PBC.

Conclusion

Conclusion. Autoimmune liver lesions are detected in 28.6% of ACA-positive patients with pSS, most (41.2%) of them develop epitheliitis of the biliary ducts as pSS manifestation or a combination of pSS with PBC (with the same frequency), less often PBC / AIH cross syndrome is diagnosed. PBC / pSS-related epitheliitis of the biliary ducts in ACA-positive patients is characterized by a slowly progressive asymptomatic course in most cases and rarely leads to the development of liver cirrhosis.

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The authors declare that there are no conflicts of interest present.