Primary sclerosing cholangitis: present views of its pathogenesis, diagnosis and treatment


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Abstract

Primary sclerosing cholangitis (PSC) is a chronic slowly progressive cholestatic liver disease characterized by non-purulent destructive inflammation and sclerosis of the extra- and intrahepatic bile ducts, leading to secondary biliary cirrhosis. The etiology of the disease is unknown; however, bacterial and genetic factors are presumed to be implicated. In 66-84% of cases, PSC is accompanied by inflammatory bowel diseases, such as ulcerative colitis and Crohn's disease. The diagnosis of PSC is based on the clinical presentation of the disease and on the data of magnetic resonance imaging or endoscopic retrograde cholangiography. The treatment of PSC has not been developed; there is evidence that ursodeoxycholic acid reduces cholestasis. Orthotopic liver transplantation is indicated at the stage of decompensated liver cirrhosis.

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Первичный склерозирующий холангит - современные представления о патогенезе, диагностике, лечении. - Аннотация. Первичный склерозирующий холангит (ПСХ) - хроническое медленно прогрессирующее холестатическое заболевание печени, характеризующееся негнойным деструктивным воспалением и склерозом вне- и внутрипеченочных желчных протоков, приводящее к формированию вторичного билиарного цирроза. Этиология заболевания неизвестна, однако предполагается влияние бактериальных и генетических факторов. В 66-84% наблюдений ПСХ сочетается с воспалительными заболеваниями кишечника - язвенным колитом и болезнью Крона. Диагностика ПСХ основана на клинической картине заболевания, а также на данных магнитно-резонансной или эндоскопической ретроградной холангиографии. Лечение ПСХ не разработано, есть данные об уменьшении холестаза при лечении урсодеоксихолевой кислотой. На стадии декомпенсированного цирроза печени показано выполнение ортотопической трансплантации печени.
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