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Publications

Journal Article Ahead of print

PubMed: 42714609 DOI: 10.1007/s00508-026-02798-6

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

Wien Klin Wochenschr

Authors: Emina Halilbasic, Elisabeth Tatscher, Elmar Aigner, Lukas Burghart, Ivo Graziadei, Stephanie Hametner-Schreil, Benedikt Silvester Hofer, Harald Hofer, Andreas Maieron, Mattias Mandorfer, Markus Peck-Radosavljevic, Benedikt Schaefer, Wolfgang Sieghart, Martin Stradner, Stefan Traussnigg, Martin Wagner, Heinz Zoller, Peter Fickert, Michael Trauner

Affiliations

  • Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
  • Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.
  • First Department of Medicine, University Clinic Salzburg, Paracelsus Medical University Salzburg, Salzburg, Austria.
  • Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
  • Klinik Ottakring, Wiener Gesundheitsverbund, Vienna, Austria.
  • Department of Internal Medicine, Academic Teaching Hospital, Hall, Austria.
  • Department of Gastroenterology and Hepatology, Ordensklinikum Linz Barmherzige Schwestern, Linz, Austria.
  • Department of Internal Medicine I, Gastroenterology and Hepatology, Rheumatology, Endocrinology and Diabetology, Klinikum Wels-Grieskirchen, Wels, Austria. Clinical Research Institute for Inflammation Medicine, Johannes Kepler University, Linz, Austria.
  • Department of Internal Medicine 2, Gastroenterology & Hepatology, University Hospital, St. Pölten, Austria.
  • Karl Landsteiner University of Health Sciences, Krems, Austria.
  • Innere Medizin und Gastroenterologie (IMuG), Klinikum Klagenfurt, Klagenfurt am Wörthersee, Austria.
  • Department of Internal Medicine I, Gastroenterology, Hepatology, Endocrinology and Metabolism, Medical University of Innsbruck, Innsbruck, Austria.
  • Imed19-Privat, Private Clinical Research Center, Vienna, Austria.
  • Division of Rheumatology and Immunology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.
  • Dr Brunner & Dr Traussnigg - Fachärzte für Innere Medizin OG, Internisten am Elterleinplatz, Vienna, Austria.
  • Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.

Abstract

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.

MeSH

Cholangitis, Gastroenterology, Antibodies, Liver, Diagnosis, Therapeutics, Peroxisome Proliferator-Activated Receptors