vtioВестник трансплантологии и искусственных органовRussian Journal of Transplantology and Artificial Organs1995-1191Academician V.I.Shumakov National Medical Research Center of Transplantology and Artificial Organs", Ministry of Health of the Russian Federation10.15825/1995-1191-2021-1-162-170vtio-1319Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSПервичный билиарный холангитPrimary biliary cholangitisИльинскийИ. М.IljinskyI. M.

Ильинский Игорь Михайлович.

123182, Москва, ул. Щукинская, д. 1.

Тел. (999) 877-60-95

Igor Iljinsky.

1, Shchukinskaya str., Moscow, 123182.

Phone: (999) 877-60-95

iiljinsky@mail.ruЦирульниковаО. М.TsirulnikovaO. M.

123182, Москва, ул. Щукинская, д. 1.

1, Shchukinskaya str., Moscow, 123182.

Национальный медицинский исследовательский центр трансплантологии и искусственных органов имени академика В.И. Шумакова Минздрава РоссииРоссияShumakov National Medical Research Center of Transplantology and Artificial OrgansRussian FederationНациональный медицинский исследовательский центр трансплантологии и искусственных органов имени академика В.И. Шумакова Минздрава России; Первый Московский государственный медицинский университет имени И.М. Сеченова Минздрава России (Сеченовский университет)РоссияShumakov National Medical Research Center of Transplantology and Artificial Organs; Sechenov UniversityRussian Federation202110042021231162170Copyright © Ильинский И.М., Цирульникова О.М., 20212021Ильинский И.М., Цирульникова О.М.Iljinsky I.M., Tsirulnikova O.M.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.transpl.ru/vtio/article/view/1319

Первичный билиарный холангит (ПБХ), ранее известный как первичный билиарный цирроз, является органоспецифическим аутоиммунным заболеванием, которым страдают преимущественно женщины среднего возраста и которое не встречается у детей. Распространенность ПБХ различается в зависимости от географического расположения страны. За последние 30 лет отмечен рост заболеваемости и одновременно достигнут значительный прогресс в понимании патогенеза ПБХ благодаря развитию инновационных технологий в молекулярной биологии, иммунологии и генетике. Наличие антимитохондриальных антител и холестаз при биохимическом анализе достаточны для постановки диагноза, без необходимости проведения биопсии печени. Мишенями при ПБХ являются мелкие и средние желчные протоки. При первой стадии заболевания наблюдается гранулематозная деструкция желчных протоков; при второй - потеря желчных протоков, их пролиферация, увеличение размеров портальных трактов с хроническим воспалением; при третьей - фиброз с образованием септ, потеря желчных протоков и холестаз; при четвертой стадии - цирроз печени. Ранее выживаемость пациентов с ПБХ составляла от 7,5 до 16 лет, но она значительно повысилась при лечении урсодезоксихолевой кислотой и обетихолевой кислотой. При отсутствии эффекта от лечения и развитии терминальной стадии печеночной недостаточности проводят трансплантацию печени.

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease predominantly affecting middle-aged women. It does not occur in children. PBC prevalence varies depending on the geographic location of the country. Over the past 30 years, there has been an increased incidence of PBC, while significant progress has been made in understanding the pathogenesis of PBC due to the development of innovative technologies in molecular biology, immunology and genetics. The presence of antimitochondrial antibodies and cholestasis on biochemical analysis is sufficient to make a diagnosis, without the need for liver biopsy. Small- and medium-sized bile ducts are the targets of PBC. In the first stage of the disease, granulomatous destruction of the bile ducts occurs; in the second stage, loss of bile ducts, their proliferation, increased size of the portal tracts with chronic inflammation; in the third stage - fibrosis with septal formation, loss of bile ducts and cholestasis; in the fourth stage - liver cirrhosis. Previously, the survival rate of PBC patients ranged from 7.5 to 16 years. However, it has improved significantly with ursodeoxycholic acid and obeticholic acid treatment. If there is no effect from treatment and end-stage liver failure sets in, liver transplantation is performed.

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