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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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2.

001-es BibID:BIBFORM135088
Első szerző:Horst, Ludwig Jesse
Cím:6-Mercaptopurine vs. Mycophenolate Mofetil in Autoimmune Hepatitis and Azathioprine Intolerance : a Multicenter Study / Horst Ludwig Jesse, Fierenz Alexander, Canhao Bernardo, Efe Cumali, Díaz-González Álvaro, Hartl Johannes, Hübener Sina, Kovats Patricia Julianna, Liberal Rodrigo, Lohse Ansgar Wilhelm, Londono Maria-Carlota, Lytvyak Ellina, Macedo Guilherme, Madaleno Joao, Munoz Beatriz Mateos, Montano-Loza Aldo J., Morris Sean M., Pallotta Dante Pio, Papp Maria, Sebode Marcial, Trivedi Palak Jitendrakumar, Oo Ye Htun, Schramm Christoph
Dátum:2026
ISSN:1542-3565 1542-7714
Megjegyzések:Background & Aims: Azathioprine (AZA) and corticosteroids are the recommended standard therapy for autoimmune hepatitis (AIH); however, a significant proportion of patients discontinue AZA due to intolerance. Although guidelines propose 6-mercaptopurine (6-MP) and mycophenolate mofetil (MMF) as alternatives, there is a lack of data on 6-MP and no comparative studies in AIH. We aimed to compare the efficacy and safety of 6-MP and MMF as second-line therapies in patients intolerant to AZA. Methods: This multicenter retrospective cohort study involved AIH patients from eleven tertiary care centers across Europe and Canada who were intolerant to AZA and subsequently switched to either 6- MP or MMF as second-line interventions. Data on biochemical response, adverse effects, and treatment duration of second-line therapies were collected and analyzed, incorporating propensity score matching to validate the biochemical response. Results: We included 211 AIH patients (81% female; median age: 54 years (IQR: 39?63 years)) with a median follow-up of 60 months (IQR: 31?105 months). MMF was better tolerated than 6-MP (89% vs. 67%; p < 0.001). Among patients who continued second-line treatment, no statistically significant difference in complete biochemical response rates was observed between 6-MP and MMF (61% and 66%, respectively, at the last follow-up). Conclusions: Both 6-MP and MMF were capable of maintaining biochemical response in patients with AIH who were intolerant to AZA, with no clear evidence of inferiority of either treatment. While MMF was generally better tolerated, 6-MP may present a safe and effective treatment option in women of reproductive age. In addition, therapy with 6-MP enables clinicians to monitor drug metabolite levels, titrate dosages, and monitor adherence.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
AIH
Second-line therapy
Azathioprine intolerance
Immunosuppression
Megjelenés:Clinical Gastroenterology and Hepatology. - [Epub ahead of print] (2026). -
További szerzők:Fierenz, Alexander Canhão, Bernardo Efe, Cumali Díaz-González, Álvaro Hartl, Johannes Hübener, Sina Kováts Patrícia (1995-) Liberal, Rodrigo Lohse, Ansgar W. Londono, Maria-Carlota Lytvyak, Ellina Macedo, Guilherme Madaleno, João Munoz, Beatriz Mateos Montano-Loza, Aldo J. Morris, Sean M. Pallotta, Dante Pio Papp Mária (1975-) (belgyógyász, gasztroenterológus) Sebode, Marcial Trivedi, Palak Jitendrakumar Oo, Ye Htun Schramm, Christoph
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3.

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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4.

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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5.

001-es BibID:BIBFORM120681
035-os BibID:(wos)001272384000001 (Scopus)85201927456
Első szerző:Weltzsch, Jan Philipp
Cím:Optimizing thiopurine therapy in autoimmune hepatitis : a multi-center study on monitoring metabolite profiles and co-therapy with allopurinol / Jan Philipp Weltzsch, Claudius Bartel, Moritz Waldmann, Thomas Renné, Stephanie Schulze, Benedetta Terziroli Beretta-Piccoli, Papp Maria, Ye Oo, Vincenzo Ronca, Marcial Sebode, Ansgar Lohse, Christoph Schramm, Johannes Hartl
Dátum:2024
ISSN:0270-9139
Megjegyzések:Background & aims: In autoimmune hepatitis (AIH), achieving complete biochemical remission (CBR) with current weight-based thiopurine dosing is challenging. We investigated whether patients could be stratified regarding CBR according to a target range of thiopurine metabolites. Moreover, we explored the effects of azathioprine dosage increases and co-therapy of allopurinol with low-dose thiopurines on metabolite profiles and treatment response. Approach & results: The relation between metabolites and treatment response was assessed in 337 individuals from four European centers. In a global, cross-sectional analysis, active metabolites 6-thioguanin nucleotides (6TGN) were similar in those with and without CBR. However, analyzing patients with sequential measurements over 4 years (N=146) revealed higher average 6TGN levels in those with stable CBR (260 pmol/0.2ml) compared to those failing to maintain CBR (181 pmol/0.2ml;p=0.0014) or never achieving CBR (153 pmol/0.2ml;p<0.0001), with an optimal 6TGN-cutoff of ?223 pmol/0.2ml (sensitivity: 76%, specificity: 78%). Only 42% exhibited 6TGN ?223 pmol/0.2ml following weight-based dosing, as doses weakly correlated with 6TGN but with 6-methylmercaptopurine (6MMP), a metabolite associated with toxicity. Azathioprine dose increases led to preferential 6MMP formation (+127% vs. 6TGN +34%;p<0.0001). Conversely, adding allopurinol to thiopurines in difficult-to-treat patients (N=36) raised 6TGN (168?321 pmol/0.2ml;p<0.0001) and lowered 6MMP (2125?184 pmol/0.2ml;p<0.0001), resulting in improved transaminases in all patients and long-term CBR in 75%. Conclusions: Maintaining CBR in AIH was associated with 6TGN ?223 pmol/0.2ml. For patients who fail to achieve CBR and therapeutic 6TGN levels despite thiopurine dose increase due to preferential 6MMP formation, co-medication of allopurinol alongside low-dose thiopurines represents an efficient alternative.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Hepatology. - 80 : 5 (2024), p. 1026-1040. -
További szerzők:Bartel, Claudius Waldmann, Moritz Renné, Thomas Schulze, Stephanie Beretta-Piccoli, Benedetta Terziroli Papp Mária (1975-) (belgyógyász, gasztroenterológus) Oo, Ye Ronca, Vincenzo Sebode, Marcial Lohse, Ansgar W. Schramm, Christoph Hartl, Johannes
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