Austrian Society of Gastroenterology and Hepatology (ÖGGH) consensus on primary biliary cholangitis

Summary This consensus document of the Austrian Society of Gastroenterology and Hepatology (ÖGGH) is intended to provide practical guidance for the management of individuals with primary biliary cholangitis (PBC). PBC is a chronic inflammatory, autoimmune-mediated disease of the intrahepatic bile ducts that can lead to fibrosis and ultimately cirrhosis. Middle-aged women are significantly more frequently affected than men. The pathogenesis is currently not fully understood. Based on the presence of disease-specific autoantibodies, it is classified as an autoimmune liver disease, although a combination of genetic predisposition and environmental factors contribute to disease development and progression. The diagnosis of PBC is based on a cholestatic enzyme pattern together with the presence of anti-mitochondrial antibodies (AMA) or PBC-specific anti-nuclear antibodies (sp100, gp210). A liver biopsy is rarely required to establish the diagnosis; exceptions are the suspicion of a PBC autoimmune hepatitis (AIH) variant syndrome or the absence of the abovementioned antibodies. The therapeutic goal is to reduce cholestatic injury thereby preventing disease progression and to reduce symptoms. Approximately 60–70% of patients achieve clinical and biochemical remission with first-line treatment, i.e., ursodeoxycholic acid (UDCA). Recently, the therapeutic paradigm has shifted from achieving certain predefined response criteria to a normalization of alkaline phosphatase (ALP) together with a low-normal bilirubin level, as the latter was linked to improved outcomes in some subgroups. For patients who do not sufficiently respond to UDCA, the newly approved peroxisome proliferator-activated receptor (PPAR) agonists elafibranor and seladelpar, as well as bezafibrate ( off-label use), should be used as a combination treatment with UDCA. In patients with decompensated cirrhosis, liver transplantation has been associated with good long-term outcomes, albeit disease recurrence occurs in up to 50% by 15 years.