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001-es BibID:	BIBFORM132792
035-os BibID:	(Scopus)105019565415 (WoS)001598867700001
Első szerző:	Gerussi, Alessio
Cím:	Trends in Primary Biliary Cholangitis : prospective Cohort Study from the European Reference Network Registry (R-LIVER) / A. Gerussi, E. Nofit, D. P. Bernasconi, C. Kroll, F. Groß, I. Schregel, A. Marini, L. Cristoferi, F. Malinverno, M. Papp, G. Dalekos, E. Rigopoulou, M. K. Janik, P. Milkiewitz, H. Ytting, A. Leburgue, F. Braun, C. Casar, M. Sebode, A. Lohse, B. Hansen, M. Carbone, C. Schramm, P. Invernizzi
Dátum:	2025
ISSN:	2050-6406 2050-6414
Megjegyzések:	Background and Aims: The European Reference Network on Hepatological Diseases (ERN RARE LIVER) is a Europe-wide network for centers of excellence in the management of rare liver diseases. We aimed to evaluate the current diagnostic and therapeutic trends of primary biliary cholangitis (PBC). Methods: Prospective data of PBC cases diagnosed from 2017 to March 2024 were extracted from the R-LIVER registry of ERN-RARE LIVER. Cases without two follow-ups within 24 months were excluded from the treatment analysis. Biochemical response according to Toronto criteria and normalization of alkaline phosphatase (ALP) values after 12 months of Ursodeoxycholic Acid (UDCA) were evaluated. Results: This study included 327 incident cases from six centers. Median age was 56 years, 89.3% were female. At the time of diagnosis, median values of ALP were 1.37 x ULN, and median bilirubin was 0.49 x ULN. Transient elastography (TE) was performed in 230 patients (70.3%) at baseline, median liver stiffness was 6.2 kPa. Out of 316 subjects, treatment with UDCA was started in 312 patients (98.7%); 246 (85.1%) achieved ALP values < 1.67 x ULN at 12 months. Normalization of ALP values occurred in 143 subjects (49.5%) at 12 months. Among 43 patients with inadequate response, 18 (41.9%) were treated with second-line therapies, and had worse liver biochemistry at baseline. Conclusion: In the current era, patients with PBC are diagnosed at an early stage using non-invasive methods and are almost all treated with UDCA. The biochemical response rate is 85.1%, but use of second-line therapies for inadequate responders remains suboptimal.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk liver autoimmunity epidemiology UDCA cholestasis
Megjelenés:	United European Gastroenterology Journal. - 13 : 10 (2025), p. 1955-1963. -
További szerzők:	Nofit, E. Bernasconi, D. P. Kroll, Claudia Groß, F. Schregel, Ida Marini, A. Cristoferi, Laura Malinverno, F. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Dalekos, George N. Rigopoulou, E. Janik, Maciej K. Milkiewicz, Piotr Ytting, Henriette Leburgue, Angela Braun, F. Casar, Christian Sebode, Marcial Lohse, Ansgar W. Hansen, Barbara C. Carbone, Marco Schramm, Christoph Invernizzi, Pietro
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001-es BibID:	BIBFORM090567
035-os BibID:	(WoS)000654740300009 (Scopus)85102488004
Első szerző:	Marjot, Thomas
Cím:	SARS-CoV-2 infection in patients with autoimmune hepatitis / Thomas Marjot, Gustav Buescher, Marcial Sebode, Eleanor Barnes, Alfred S. Barritt IV, Matthew J. Armstrong, Luke Baldelli, James Kennedy, Carolyn Mercer, Ann-Kathrin Ozga, Christian Casar, Christoph Schramm, Andrew M. Moon, Gwilym J. Webb, Ansgar W. Lohse, Collaborators of ERN RARE-LIVER / COVID-Hep / SECURE-Cirrhosis
Dátum:	2021
ISSN:	0168-8278
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk autoimmune liver disease autoimmune hepatitis SARS-CoV-2 COVID-19 immunosuppression
Megjelenés:	Journal Of Hepatology. - 74 : 6 (2021), p. 1335-1343. -
További szerzők:	Buescher, Gustav Sebode, Marcial Barnes, Eleanor Barritt, Alfred S. IV Armstrong, Matthew J. Baldelli, Luke Kennedy, James Mercer, Carolyn Ozga, Ann-Kathrin Casar, Christian Schramm, Christoph Moon, Andrew M. Webb, Gwilym J. Lohse, Ansgar W. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Balogh Boglárka (1993-) (belgyógyász) Collaborators of ERN RARE-LIVER / COVID-Hep / SECURE-Cirrhosis
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001-es BibID:	BIBFORM121373
035-os BibID:	(WoS)001273414600001 (Scopus)85199139220
Első szerző:	Schregel, Ida
Cím:	Unmet needs in autoimmune hepatitis : results of the prospective multicentre European Reference Network Registry (R-LIVER) / Schregel Ida, Papp Maria, Sipeki Nora, Kovats Patricia J., Taubert Richard, Engel Bastian, Campos-Murguia Alejandro, Dalekos George N., Gatselis Nikolaos, Zachou Kalliopi, Milkiewicz Piotr, Janik Maciej K., Raszeja-Wyszomirska Joanna, Ytting Henriette, Braun Felix, Casar Christian, Sebode Marcial, Lohse Ansgar W., Schramm Christoph, European Reference Network (ERN) RARE-LIVER
Dátum:	2024
ISSN:	1478-3223
Megjegyzések:	The European Reference Network on Hepatological Diseases (ERN RARE-LIVER) launched the prospective, multicentre, quality-controlled R-LIVER registry on rare liver diseases. The aim of this study was to assess the presentation and outcome of autoimmune hepatitis (AIH) after one year of treatment. Methods Data was prospectively collected at the time of diagnosis, and after 6- and 12-months follow-up. Complete biochemical response (CBR) was defined as normalization of alanine aminotransferase (ALT) and immunoglobulin G (IgG) serum levels. Results 231 patients from six European centres were included in the analysis. After six months of treatment 50% (106/212), and after 12 months 63% (131/210) of patients reached CBR with only 27% (56/211) achieving a steroid-free CBR within the first year. Overall, 16 different treatment regimens were administered. Change of treatment, mostly due to intolerance, occurred in 30.4% within the first six months. In multivariate analysis, younger age at diagnosis (OR = 1.03 [95%CI 1.01 ? 1.05]; p = .007), severe fibrosis (OR 0.38 [95% 0.16 ? 0.89], p = .026) and change of treatment within the first six months (OR 0.40 [95% CI 0.2 ? 0.86]; p = .018) were associated with a lesser chance of ALT normalization at 12 month-follow-up. The landscape of AIH-treatment in Europe is highly heterogenous, even between expert centres. The results from this first European multicentre prospective registry reveal several unmet needs, highlighted by the overall low rates of CBR and the frequent failure to withdraw corticosteroids.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk Autoimmune Hepatitis treatment regime complete biochemical response immunosuppression remission
Megjelenés:	Liver International. - 44 : 10 (2024), p. 2687-2699. -
További szerzők:	Papp Mária (1975-) (belgyógyász, gasztroenterológus) Sipeki Nóra (1987-) (általános orvos) Kováts Patrícia (1995-) Taubert, Richard Engel, Bastian Campos-Murguia, Alejandro Dalekos, George N. Gatselis, Nikolaos K. Zachou, Kalliopi Milkiewicz, Piotr Janik, Maciej K. Raszeja-Wyszomirska, Joanna Ytting, Henriette Braun, Felix Casar, Christian Sebode, Marcial Lohse, Ansgar W. Schramm, Christoph European Reference Network (ERN) RARE-LIVER
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001-es BibID:	BIBFORM123332
035-os BibID:	(scopus)85202766719
Első szerző:	Stern, Louisa
Cím:	Efficacy and safety of palliative treatment in patients with autoimmune liver disease-associated hepatocellular carcinoma / Stern Louisa, Schmidt Constantin, Kocheise Lorenz, Joerg Vincent, Casar Christian, Walter Aurélie, Drenth Joost P. H., Papp Maria, Gatselis Nikolaos K., Zachou Kalliopi, Pinter Matthias, Scheiner Bernhard, Vogel Arndt, Kirstein Martha M., Finkelmeier Fabian, Waidmann Oliver, Weinmann Arndt, Milkiewicz Piotr, Thorburn Douglas, Halliday Neil, Lleo Ana, Huber Samuel, Dalekos George N., Lohse Ansgar W., Wege Henning, von Felden Johann, Schulze Kornelius
Dátum:	2024
ISSN:	1665-2681
Megjegyzések:	Introduction and Objectives Autoimmune liver diseases (AILD) are rare causes hepatocellular carcinoma (HCC). and data on the efficacy and tolerability of anti-tumour therapies are scarce. This pan-European study aimed to assess outcomes in AILD-HCC patients treated with tyrosine kinase inhibitors (TKIs) or transarterial chemoembolization (TACE) compared with patients with more common HCC etiologies, including viral, alcoholic or non-alcoholic fatty liver disease. Materials and Methods 107 patients with HCC-AILD (AIH:55; PBC:52) treated at 13 European centres between 1996 and 2020 were included. 65 received TACE and 28 received TKI therapy. 43 (66%) were female (median age 73 years) with HCC tumour stage BCLC A (34%), B (46%), C (9%) or D (11%). For each treatment type, propensity score matching was used to match AILD to non-AILD-HCC on a 1:1 basis, yielding in a final cohort of 130 TACE and 56 TKI patients for comparative analyses of median overall survival (mOS) and treatment tolerability. Results HCC-AILD patients showed comparable mOS to controls for both TACE (19.5 vs 22.1 months, p=0.9) and TKI (15.4 vs 15.1 months, p=0.5). Adverse events were less frequent in AILD-HCC patients than controls (33% vs 62%, p=0.003). For TKIs, there were no significant differences in adverse events (73% vs. 86%, p=0.2) or interruption rates (44% vs. 36%, p=0.7). Conclusions In summary, this study demonstrates comparable mOS for AILD-HCC patients undergoing local and systemic treatments, with better tolerability than HCC of other causes. TKIs remain important therapeutic options for AILD-HCC patients, particularly given their exclusion from recent immunotherapy trials.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk TACE AILD TKI HCC AIH PBC
Megjelenés:	Annals of Hepatology. - 29 : 6 (2024), p. 1-6. -
További szerzők:	Schmidt, Constantin Kocheise, Lorenz Joerg, Vincent Casar, Christian Walter, Aurélie Drenth, Joost P. H. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Gatselis, Nikolaos K. Zachou, Kalliopi Pinter, Matthias Scheiner, Bernhard Vogel, Arndt Kirstein, Martha M. Finkelmeier, Fabian Waidmann, Oliver Weinmann, Arndt Milkiewicz, Piotr Thorburn, Douglas Halliday, Neil Lleo, Ana Huber, Samuel Dalekos, George N. Lohse, Ansgar W. Wege, Henning von Felden, Johann Schulze, Kornelius
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