Sub-optimal therapy of patients with primary biliary cholangitis (PBC) in the real-life stetting of the German PBC cohort
Year: 2024
Type: article
Study design: Observational Study
Abstract: Real-world data on the management of patients with primary biliary cholangitis (PBC) are so far scarce in Germany. Therefore, we aimed to establish a nationwide registry and describe the clinical characteristics and therapy of PBC patients.Three different cohorts defined as ursodeoxycholic acid (UDCA) responders, as inadequate responders according to Paris II criteria, and as newly diagnosed patients were prospectively recruited.This manuscript includes the baseline data of the project.In total, 33/77 (43%) contacted centres (58% of university hospitals, 38% of non-university hospitals, and 24% of private practices) recruited 515 patients including 204 UDCA responders, 221 inadequate responders to UDCA, and 90 newly diagnosed patients.All patients were treated with UDCA; however, a UDCA dosage below the recommended dosage of 13 mg/kg/d was observed in 38.5% of individuals after 12 months of treatment. UDCA dosages were lower in nonacademic compared to academic centres.Only 75/219 (38.5%) of inadequate responders to UDCA received a second-line therapy with obeticholic acid (OCA) and/or bezafibrate (BZF). OCA (13% vs. 4.5%) and BZF (14% vs. 6.5%) were significantly more often prescribed by academic vs. nonacademic centres.Pruritus (27% vs. 15.5%), fatigue (23% vs. 4.5%), and sicca syndrome (14% vs. 1%) were significantly more often reported by academic centres.The German PBC registry could be established, which indicates suboptimal therapy in a relevant proportion of patients and shows significant differences between academic and nonacademic centres. Results are fundamental to improving clinical management at different levels of care.
DOI: 10.1055/a-2382-7720
Authors: Johannes Wiegand, Annegret Franke, Tobias Müller, Kerstin Stein, Heike Bantel, Rainer Günther, Gerald Denk, Philipp A. Reuken, Jörn M. Schattenberg, Uwe Naumann, Tobias Böttler, Andreas Weber, Stefan Zeuzem, Matthias Hinz, Robin Greinert, Christoph Berg, Thaddäus Till Wissniowski, Karl-Georg Simon, Jonel Trebicka, Rüdiger Behrens, Harald Grümmer, Wolf Peter Hofmann, Nektarios Dikopoulos, Christoph Sarrazin, Elke Roeb, Andreas E. Kremer, Marion Muche, Marc Ringelhan, Andreas Teufel, Patrick Michl, Verena Keitel, Jens U. Marquardt, Achim Kautz, Frank Tacke, Katja Piotrowski, Nicole Köppe-Bauernfeind, Christian Trautwein, Thomas Berg
Institutions: University Hospital Leipzig, Leipzig University, Charité - Universitätsmedizin Berlin, Medizinische Hochschule Hannover, University Hospital Schleswig-Holstein, LMU Klinikum, Ludwig-Maximilians-Universität München, Jena University Hospital, Johannes Gutenberg University Mainz, University Medical Center of the Johannes Gutenberg University Mainz, University of Freiburg, University Medical Center Freiburg, Nuremberg Hospital, Goethe University Frankfurt, University Hospital Frankfurt, University Hospital in Halle, University of Tübingen, Klinikum Chemnitz, University of Münster, St. Josefs Hospital, Justus-Liebig-Universität Gießen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Universitätsklinikum Erlangen, University Hospital Zurich, Humboldt-Universität zu Berlin, Freie Universität Berlin, TUM Klinikum, Technical University of Munich, Heidelberg University, Medizinische Fakultät Mannheim, University Hospital Heidelberg, University Hospital Magdeburg, Otto-von-Guericke-Universität Magdeburg, Leibniz Research Centre for Working Environment and Human Factors
Language: English
Topic: Liver Diseases and Immunity
Subfield: Hepatology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W4402163466