Sub-optimal therapy of patients with primary biliary cholangitis (PBC) in the real-life stetting of the German PBC cohort.

Autor:	Wiegand J; Division of Hepatology, Department of Medicine II, University Medical Centre, Leipzig, Germany., Franke A; Clinical Trial Centre of Medical Faculty, University of Leipzig, Leipzig, Germany., Müller T; Department of Hepatology and Gastroenterology, Campus Virchow-Klinikum (CVK) and Campus Charité Mitte (CCM), Charité - Universitätsmedizin Berlin, Berlin, Germany., Stein K; Practice of Hepatology Magdeburg, Magdeburg, Germany., Bantel H; Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Hannover, Germany., Günther R; Division of Hepatology, Department of Medicine I, Universitätsklinikum Schleswig-Holstein, Kiel, Germany., Denk G; Department of Medicine II and Transplantation Center, University Hospital, LMU Munich, Munich, Germany., Reuken PA; Department of Internal Medicine IV (Gastroenterology, Hepatology, Infectious diseases), Jena University Hospital, Jena, Germany., Schattenberg JM; I. Department of Medicine, Metabolic Liver Research Program, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany., Naumann U; UBN Private Practice, Berlin, Germany., Böttler T; Department of Medicine II, University Hospital Freiburg, Freiburg, Germany., Weber A; Department of Internal Medicine 6 Gastroenterology, Hepatology, Endocrinology, Hospital Nuremberg, Nuremberg, Germany., Zeuzem S; Department of Medicine, Goethe University, University Hospital, Frankfurt am Main, Germany., Hinz M; Private Practice for Gastroenterology Herne, Herne, Germany., Greinert R; Department of Gastroenterology, University Hospital Halle, Halle, Germany., Berg C; Department of Internal Medicine I, University of Tübingen, Tübingen, Germany., Wissniowski TT; Zentrum Innere Medizin II, Klinikum Chemnitz gGmbH, Chemnitz, Germany., Simon KG; Practice for Gastroenterology, Leverkusen, Germany., Trebicka J; Department of Internal Medicine B, University of Münster, Münster, Germany., Behrens R; Private Practice Halle, Halle, Germany., Grümmer H; Practice of Internal Medicine/Gastroenterology, Potsdam, Germany., Hofmann WP; Gastroenterology at Bayerischer Platz, Berlin, Germany., Dikopoulos N; Practice for Gastroenterology & Internal Medicine Ludwig & Dikopoulos, Dornstadt, Germany., Sarrazin C; Medizinische Klinik 2, St. Josef Hospital, Wiesbaden, Germany., Roeb E; Gastroenterology, University of Gießen, Gießen, Germany., Kremer AE; Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany.; Department of Gastroenterology and Hepatology, University Hospital Zürich, Zurich, Switzerland., Muche M; Department of Gastroenterology, Infectious Diseases, and Rheumatology (Campus Benjamin Franklin), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany., Ringelhan M; Second Medical Department, University Hospital rechts der Isar, Technical University Munich, School of Medicine, Munich, Germany., Teufel A; Department of Medicine II, Division of Hepatology, Division of Bioinformatics, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany., Michl P; University Hospital Heidelberg, Dept. of Internal Medicine IV, Heidelberg, University of Heidelberg, Heidelberg, Germany., Keitel V; University Hospital Magdeburg, Otto-von Guericke University Magdeburg, Magdeburg, Germany., Marquardt JU; Campus Lübeck, First Department of Medicine, University Hospital Schleswig-Holstein, Lübeck, Germany., Kautz A; Kautz5 gUG, Köln, Germany., Tacke F; Department of Hepatology and Gastroenterology, Campus Virchow-Klinikum (CVK) and Campus Charité Mitte (CCM), Charité - Universitätsmedizin Berlin, Berlin, Germany., Piotrowski K; Clinical Trial Centre of Medical Faculty, University of Leipzig, Leipzig, Germany., Köppe-Bauernfeind N; Clinical Trial Centre of Medical Faculty, University of Leipzig, Leipzig, Germany., Trautwein C; Leibniz-Institut für Arbeitsforschung an der TU Dortmund, Dortmund, Germany., Berg T; Division of Hepatology, Department of Medicine II, University Medical Centre, Leipzig, Germany.
Jazyk:	angličtina
Zdroj:	Zeitschrift fur Gastroenterologie [Z Gastroenterol] 2024 Nov; Vol. 62 (11), pp. 1931-1942. Date of Electronic Publication: 2024 Sep 03.
DOI:	10.1055/a-2382-7720
Abstrakt:	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. Competing Interests: JW: Lecturer and advisory board member for Intercept/Advanz Pharma, GSK, Ipsen TM: Ssupported by the German Research Foundation Grants MU 2864/1-3 and MU 2864/3- 1.KS: Receipt of speaker´s honoraria or advisory board: Gilead, Intercept/Advanz Pharma, Abbvie, Falk, Novo Nordisk HB: Consultant: Intercept/Advance Pharma, Ipsen GD: Consultant / speaker: AbbVie, Advanz/Intercept, Alexion, Falk Foundation, Gilead, Novartis, Orphalan, Univar PAR: Consulting and lectures fees: Astra Zeneca, BMS, Boston Scientific, CSL Behring, Gilead, Pfizer, Abbvie, Norgine JMS: Consultant: Astra Zeneca, Apollo Endosurgery, Bayer, Boehringer Ingelheim, BMS, Gilead Sciences, GSK, Intercept Pharmaceuticals, Ipsen, Inventiva Pharma, Madrigal, MSD, Northsea Therapeutics, Novartis, Novo Nordisk, Pfizer, Roche, Sanofi, Siemens Healthineers. Research Funding: Gilead Sciences, Boehringer Ingelheim, Siemens Healthcare GmbH. Stock Options: AGED diagnostics, Hepta Bio. Speaker Honorarium: Boehringer Ingelheim, Echosens, MedPublico GmbH, Novo Nordisk, Madrigal Pharmaceuticals, Histoindex, MedPublico GmbH SZ: Speakers bureau and/or consultancy: Abbvie, BioMarin, Boehringer Ingelheim, Gilead, GSK, Intercept, Ipsen, Janssen, Madrigal, MSD/Merck, NovoNordisk, SoBi, Theratechnologies KGS: Consultant: Advance Pharma, Speaker Honorarium: AbbVie, GileadJT has received speaking and/or consulting fees from Versantis, Gore, Boehringer-Ingelheim, Falk, Grifols, Genfit and CSL Behring. WPH: Consultant or Speaker Honorarium: Intercept /Advanz, Ipsen, NovoNordisk, Gilead, Abbvie, Norgine CS: Consultant, Study support or Speaker Honorarium: Calliditas, Falk, Intercept/Advanz, Ipsen, Mirum ER: Receipt of honoraria or consultation fees/advisory board: Abbvie, Amgen, Intercept, Medac, Merz, Norgine, Falk Foundation, Gilead, Pfizer, Repha, Takeda AEK: Research grant: Intercept. Speakers bureau: Abbvie, Advanz, AOP Orphan, Bayer, BMS, CMS, CymaBay, Falk, Gilead, GSK, Intercept, Ipsen, Newbridge, Novartis, Lilly, Mirum, MSD, Roche, Zambon. Consultant: Abbvie, Advanz, Alentis, AlphaSigma, AstraZenca, Avior, Bayer, BioNTech, CymaBay, Eisai, Escient, Falk, FMC, Gilead, GSK, Guidepoint, Intercept, Ipsen, Mirum, Medscape, MSD, Myr, Roche, Takeda, Viofor MR: Consultant, or Speaker Honorarium: Intercept/Advanz, Gilead, Abbvie AT: Speaker Honorarium: Intercept/Advanz VK: Consultant Astra Zeneca, Speaker’s Honoraria from AbbVie, Gilead, Falk, Mirum, Albireo/Ipsen, Merck, MedUpdate GmbH, Sanofi, CSL Behring ER: Receipt of honoraria or consultation fees/advisory board: Abbvie, Amgen, Intercept, Medac, Merz, Norgine, Falk Foundation, Gilead, Pfizer, Repha, Takeda FT: Research grant: Allergan, BMS, Inventiva, Gilead. Speakers bureau: Gilead, Abbvie, Falk, Merz, Orphalan, Advanz. Consultant: Allergan, AstraZeneca, Gilead, Abbvie, Alnylam, BMS, Intercept / Advanz, Inventiva, Pfizer, Novartis, Novo Nordisk, Sanofi. CT: Receipt of honoraria or consultation fees/advisory board: Intercept/Advanz Pharma TB: Receipt of grants/research supports: Abbvie, BMS, Gilead, MSD/Merck, Humedics, Intercept, Merz, Norgine, Novartis, Orphalan, Sequana Medical; Receipt of honoraria or consultation fees/advisory board: Abbvie, Alexion, Albireo, Bayer, Gilead, GSK, Eisai, Enyo Pharma, HepaRegeniX GmbH, Humedics, Intercept, Ipsen, Janssen, MSD/Merck, Novartis, Orphalan, Roche, Sequana Medical, SIRTEX, SOBI, and Shionogi; Participation in a company sponsored speaker’s bureau: Abbvie, Advance Pharma, Alexion, Albireo, Bayer, Gilead, Eisai, Falk Foundation, Intercept, Ipsen, Janssen, MedUpdate GmbH, MSD/Merck, Novartis, Orphalan, Sequana Medica, SIRTEX, and SOBI Nothing to disclose: AF, RG, UN, TB, AW, MH, RG, CB, TTW, RB, HG, ND, MM, PM, JUM, AK, KP, NKB (Thieme. All rights reserved.)
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