Первичный билиарный холангит: новая дефиниция заболевания, актуальные аспекты диагностики и лечения. Обзор литературы

Abstract

Primary biliary cholangitis (PBc) is a chronic autoimmune cholestatic liver disease characterized by T-lymphocyte-mediated damage to intrahepatic biliary epithelial cells and the presence of a specific serological marker, antimitochondrial antibodies, and which, if left untreated, leads to end-stage biliary cirrhosis. PBc remains a major public health problem. until recently, PBc was referred to as "primary biliary cirrhosis", but the disease is often diagnosed in the pre-cirrhotic phase, when there is no formed cirrhosis yet, but there is only a threat of its development. The change in the definition of the disease harmoniously fits into the framework of the concept of preventive medicine, since with timely diagnosis and adequate therapy, this phase of the disease can be extended for years and decades, but all these years the patient is “traumatized” by a prediagnosed liver cirrhosis. At the end of the previous decade, a large number of international clinical recommendations and guidelines on PBc were published. As part of the review of the current state of the PBc problem, publications from various international databases were used, with an emphasis on publications of the current decade, which note the importance of early diagnosis of the disease with an emphasis on biochemical markers of cholestasis (primarily alkaline phosphatase) and immunological markers (primarily turn antimitochondrial antibodies) and early initiation of treatment with the first-line drug – ursodeoxycholic acid. Keywords: primary biliary cholangitis, definition of the disease, epidemiology, diagnosis, alkaline phosphatase, antimitochondrial antibodies, clinic, treatment, ursodeoxycholic acid, obeticholic acid, monitoring, prognosis.