Primary Biliary Cholangitis and Ursodeoxycholic Acid: Therapy Progress and Challenges
Objective of the Review: To summarise available information about the aetiopathogenesis and clinical features of primary biliary cholangitis (PBC); to address topical issues of diagnostics, treatment and risk stratification in patients with PBC. Key Points: PBC (formerly known as primary biliary cirrhosis) is a chronic inflammatory autoimmune condition primarily affecting cholangiocytes in interlobular bile ducts.PBC is most common for middle-age women. If untreated PBC progresses to hepatic cirrhosis and hepatic failure.PBC onset and progression are a multiple-factor process where various environmental factors impact genetic, epigenetic, immunologic status of the patient. Early PBC diagnostics has improved significantly due to identification of standard serological markers - antimitochondrial antibodies. In 1980s PBC was a primary indication for liver transplantation. Currently ursodeoxycholic acid (UDCA) is the first line drug; the majority of patients with PBC treated with UDCA have normal life span. However 1 out of 3 patients does not respond to the UDCA therapy, therefore it is clinically important to assess response with biochemical tests after one year of the therapy. Conclusion: Early diagnostics and stratification of PBC patients with low and high risk of UDCA therapy tolerance are clinically significant for an individual approach to treatment and assessment of the need in additional drug therapy.