Primary Biliary Cholangitis (PBC) is a chronic inflammatory autoimmune cholestatic liver disease. Most importantly, when untreated will culminate in end-stage biliary cirrhosis. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse and risk stratification is important. Most importantly to ensure all patients receive a personalised approach to their care.
The goals of treatment and management of Primary Biliary Cholangitis are the prevention of end-stage liver disease. Also the amelioration of associated symptoms. Pharmacologic approaches in practice, to reduce the impact of the progressive nature of disease, currently include licensed therapies (ursodeoxycholic acid and obeticholic acid). And also off-label therapies (fibric acid derivatives, budesonide). This EASL Guideline summarise the evidence for the importance of a structured, life-long and individualised, approach to the care of patients with PBC. Finally, it provides a framework to help clinicians diagnose and effectively manage patients.
More on Primary Biliary Cholangitis
Primary Biliary Cholangitis (PBC; formerly known as primary biliary cirrhosis) is an important but uncommon disease that predominantly affects women. It is a globally recognized autoimmune cholestatic liver disease with several characteristics. Most importantly: cholestasis, serologic reactivity to antimitochondrial
antibodies (AMA) or specific antinuclear antibody (ANA) reactivity, with accompanying histologic evidence of chronic non-suppurative, granulomatous, lymphocytic small bile duct cholangitis. Primary Biliary Cholangitis is chronic and often progressive, resulting in end-stage liver disease and its associated complications. The youngest reported age of confirmed disease onset is 15 in a post-menarche young adult. The goal of life-long therapy is to prevent progressive liver disease. Most importantly to ameliorate diseaseassociated symptoms that reduce patient quality of life (QoL). In conclusion, data from multiple studies indicate that globally, an estimated 1 in 1,000 women over the age of 40 live with PBC.
Download the EASL Guideline for Management of Patients with Primary Biliary Cholangitis as PDF or PPT Slide Deck from the right of your screen.