GENERAL CHARACTERISTICS OF PRIMARY BILIARY CHOLANGITIS IN THE WORLD. LITERATURE REVIEW
Background: Primary biliary cholangitis (PBC, formerly known as primary biliary cirrhosis of the liver [54]) is one of the most significant diseases that is mainly verified in women. PBC is included in the group of autoimmune cholestatic liver diseases [37,3-5]. The overall prevalence and frequency of PBC remains low compared to other liver diseases. According to the organization for Organ Delivery and Transplantation, cases of PBC accounted for only 165 out of 8,250 liver transplants performed in 2018. [4,45]. The overall incidence of PBC has continued to grow since the 1980s. [4,5-8] PBC is considered a rare disease, mainly due to the small sample size and the lack of large long - term studies. Moreover, the recorded data on prevalence and morbidity often differed dramatically between studies and regions around the world (and even in different US states). [4] Aim: to reflect the general characteristics of primary biliary cholangitis in the world, as well as the results of our own observation on the basis of the Research Institute of Cardiology and Internal Diseases of the Ministry of Health of the Republic of Kazakhstan. Search strategy: The search and analysis of scientific publications in the databases and web resources MEDLINE, UptoDate, Google Scholar, Cochrane, PubMed by keywords (autoimmune liver diseases, primary biliary cholangitis, primary biliary cirrhosis) was carried out. Of all the selected articles, 62 sources were included for subsequent analysis, which meet the inclusion criteria and exclude duplication or repetition of information. As well as the results of our own observation on the basis of the Research Institute of Cardiology and Internal Diseases of the Ministry of Health in the period from 2014 to April 2021. Results: 1. PBC is one of the most significant autoimmune liver diseases, which is less common than viral hepatitis and fatty liver disease, but in recent decades there has been a steady increase in this disease. 2. The epidemiological features of PBC in the world are the predominant prevalence in women. A retrospective analysis showed that in the 1990s and early 2000s, the ratio of women to men was 9:1. In the conditions of the Research Institute of K and WB, 97.3% of 189 cases were women (184), 2.7% were men (5). The average age of the general group was 53.2 years. 3. The etiology is based on the importance of genetic predisposition, intestinal microbiota, bile acids and environmental triggers, such as smoking and infections of the genitourinary system. 4. The criteria for making a diagnosis are 2 out of 3 points: 1. Increase of cholestasis enzymes, 2. Detection of AMA and 3. Typical liver histology. 5. According to our own research conducted on the basis of the Research Institute, out of 189 recorded cases, positive AMA was verified - 82%(155), negative AMA - 18% (34).