[PRIMARY BILIARY CHOLANGITIS: THERAPEUTIC APPROACH IN THE MODERN ERA]

Primary biliary cholangitis (PBC) is a progressive, autoimmune cholestatic liver disease, predominantly affecting middle-aged women. Hallmark features include a persistent elevation of cholestatic liver enzymes, presence of anti-mitochondrial antibodies and characteristic histologic findings. PBC has a varied course of progression, ranging from mild uncomplicated disease to aggressive disease leading to cirrhosis and resulting in the need for liver transplantation. More than a half of the patients are asymptomatic, but the clinical phenotype varies, and symptoms may be debilitating and have a major impact on quality of life. The goals of PBC management are slowing disease progression, amelioration of associated symptoms and addressing complications of chronic liver disease. The introduction of ursodeoxycholic acid (UDCA) therapy and its universal use as the first-line therapy for PBC has favorably impacted long term prognosis and drastically changed the natural history and disease-related mortality. However, a substantial subpopulation of patients exhibits an incomplete response to UDCA, associated with a sustained disease progression and a poor outcome. Recently, obeticholic acid (OCA) was officially approved as an add-on treatment in patients not responding or intolerant to UDCA. Although evidence for biochemical improvement by OCA is compelling, long-term clinical impact is still under ongoing research. Novel treatment concepts and potential therapeutic options are under investigation. The current review addresses treatment aspects of PBC, while shedding light on the latest updates in patients' management and follow-up.