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1.

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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2.

001-es BibID:BIBFORM085824
Első szerző:Pape, Simon
Cím:High discontinuation rate of azathioprine in autoimmune hepatitis, independent of time of treatment initiation / Pape Simon, Gevers Tom J. G., Vrolijk Jan Maarten, van Hoek Bart, Bouma Gerd, van Nieuwkerk Carin M. J., Taubert Richard, Jaeckel Elmar, Manns Michael P., Papp Maria, Sipeki Nora, Stickel Felix, Efe Cumali, Ozaslan Ersan, Purnak Tugrul, Nevens Frederik, Kessener Dominik J. N., Kahraman Alisan, Wedemeyer Heiner, Hartl Johannes, Schramm Christoph, Lohse Ansgar W., Heneghan Michael A., Drenth Joost P. H.
Dátum:2020
ISSN:1478-3223
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Liver International. - 40 : 9 (2020), p. 2164-2171. -
További szerzők:Gevers, Tom J. G. Vrolijk, Jan Maarten van Hoek, Bart Bouma, Gerd van Nieuwkerk, Carin M. J. Taubert, Richard Jaeckel, Elmar Manns, Michael P. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Sipeki Nóra (1987-) (általános orvos) Stickel, Felix Efe, Cumali Ozaslan, Ersan Purnak, Tugrul Nevens, Frederik Kessener, Dominik J. N. Kahraman, Alisan Wedemeyer, Heiner Hartl, Johannes Schramm, Christoph Lohse, Ansgar W. Heneghan, Michael A. Drenth, Joost P. H.
Pályázati támogatás:ÚNKP-19-4
Egyéb
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3.

001-es BibID:BIBFORM081815
035-os BibID:(WoS)000552446400014 (Scopus)85084968025
Első szerző:Pape, Simon
Cím:Rapid Response to Treatment of Autoimmune Hepatitis Associated with Remission at 6 and 12 Months / Simon Pape, Tom J. G. Gevers, Jan Maarten Vrolijk, Bart van Hoek, Gerd Bouma, Carin M. J. van Nieuwkerk, Richard Taubert, Elmar Jaeckel, Michael P. Manns, Maria Papp, Nora Sipeki, Felix Stickel, Cumali Efe, Ersan Ozaslan, Tugrul Purnak, Frederik Nevens, Dominik J. N. Kessener, Alisan Kahraman, Heiner Wedemeyer, Johannes Hartl, Christoph Schramm, Ansgar W. Lohse, Joost P. H. Drenth, Michael A. Heneghan
Dátum:2020
ISSN:1542-3565 1542-7714
Megjegyzések:Background & Aims: Changes in serum levels of transaminases immediately after initiation of treatment for autoimmune hepatitis (AIH) might be associated with biochemical markers of remission and liver-related events. We assessed the outcomes of patients with vs without rapid responses to treatment of AIH in a large international cohort. Methods: We performed a retrospective cohort study, collecting data from 2 independent cohorts of adults with AIH from 12 centers in 7 countries in Europe. We collected information on patient demographics; serologic, histologic, and biochemical analyses; and treatment. We used a receiver operating characteristic curve and Youden index to calculate the optimal percentage decrease in level of aspartate aminotransferase (AST) after 8 weeks of treatment that associated with normalization of transaminase levels after 26 weeks of treatment with predniso(lo)ne (primary outcome) in the first (discovery) cohort (n = 370). We evaluated the results in the second (validation) cohort (n = 370). Secondary outcomes were liver-related death or transplantation. We performed univariate and multivariable logistic and Cox regression with correction for confounders. Results: A significant decrease in level of AST after 8 weeks of treatment was significantly associated with normalization of transaminase levels at 26 and 52 weeks ( P <.001); a decrease of more than 80% in level of AST was associated with optimal normalization. In both cohorts, rapid responders (?80% decrease in level of AST after 8 weeks) were more likely to achieve normalization of transaminases at 26 and 52 weeks when compared to non-rapid responders. Rapid responders in the discovery cohort had lower risk of liver-related death or transplantation (adjusted hazard ratio 0.18; 95% CI 0.05?0.63; P =.007), although this was not confirmed in the validation cohort. Results from measurement of alanine aminotransferase did not differ significantly from those of AST for the primary outcome. Slow responders (without normalization of transaminases after 1 year) had the highest risk of liver transplantation or liver-related death. Conclusions: In a retrospective study of patients with AIH, we found that a rapid response to treatment, based on level of AST after 8 weeks, associates with normalization of transaminase levels in the following year. Patients with a rapid response also have a lower risk of liver-related death or transplantation than patients without this rapid response.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Induction therapy,
prognostic factor,
liver enzyme,
Megjelenés:Clinical Gastroenterology and Hepatology. - 18 : 7 (2020), p. 1609-1617. -
További szerzők:Gevers, Tom J. G. Maarten Vrolijk, Jan Hoek, Bart van Bouma, Gerd Nieuwkerk, Carin M. J. van Taubert, Richard Jaeckel, Elmar Manns, Michael P. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Sipeki Nóra (1987-) (általános orvos) Stickel, Felix Efe, Cumali Ozaslan, Ersan Purnak, Tugrul Nevens, Frederik Kessener, Dominik J. N. Kahraman, Alisan Wedemeyer, Heiner Hartl, Johannes Schramm, Christoph Lohse, Ansgar W. Drenth, Joost P. H. Heneghan, Michael A.
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4.

001-es BibID:BIBFORM077050
035-os BibID:(WoS)000482217300030 (Scopus)85065040027
Első szerző:Pape, Simon
Cím:Predniso(lo)ne Dosage and Chance of Remission in Patients With Autoimmune Hepatitis / Simon Pape, Tom J. G. Gevers, Michail Belias, Ilyas F. Mustafajev, Jan Maarten Vrolijk, Bart van Hoek, Gerd Bouma, Carin M. J. van Nieuwkerk, Johannes Hartl, Christoph Schramm, Ansgar W. Lohse, Richard Taubert, Elmar Jaeckel, Michael P. Manns, Maria Papp, Felix Stickel, Michael A. Heneghan, Joost P. H. Drenth
Dátum:2019
ISSN:1542-3565 1542-7714
Megjegyzések:BACKGROUND & AIMS: Patients with autoimmune hepatitis (AIH) commonly receive induction therapy with predniso(lo)ne followed by maintenance therapy with azathioprine. European Association for Study of the Liver clinical practice guidelines advise a predniso(lo)ne dose range of 0.50-1 mg/kg/day, which leaves room for practice variation. We performed a multicenter study to determine the efficacy of different dose ranges of predniso(lo)ne induction therapy in a large European cohort of patients with AIH. METHODS: We performed a retrospective cohort study using a comparative effectiveness design. We collected data from 451 adults with AIH who began treatment from 1978 through 2017 at 9 centers in 5 European countries. We assigned patients to a high-dose group (initial predniso(lo)ne dose ?0.50 mg/kg/day; n=281) or a low-dose group (<0.50 mg/kg/day; n=170). Logistic regression was performed to determine difference in outcomes between the groups. The primary outcome was normal serum levels of transaminases at 6 months after initiation of therapy. RESULTS: There was no significant difference in rates of normalization of transaminases between the high-dose predniso(lo)ne group and the low-dose group (70.5% vs 64.7%; P=.20). After multivariable logistic regression with correction for confounders, there was no difference in the likelihood of normalization of transaminases between the groups (odds ratio, 1.21; 95% CI, 0.78 - 1.87; P=.38). Patients given an initial high dose of predniso(lo)ne received more predniso(lo)ne over time than patients started on a lower dose (median doses over 6 months: 3780 mg vs 2573 mg) (P<.01). CONCLUSIONS: In a retrospective study of patients with AIH in Europe, we found that the dose of predniso(lo)ne to induce remission in patients with AIH is less relevant than assumed. An initial predniso(lo)ne dose below 0.50 mg/kg/day substantially decreases unnecessary exposure to predniso(lo)ne in patients with AIH.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
autoimmune hepatitis
EASL guidelines
ALT
AST
IgG
corticosteroid
induction therapy
cirrhosis
prednison
prednisolon
Megjelenés:Clinical Gastroenterology and Hepatology. - 17 : 10 (2019), p. 2068-2075.e2. -
További szerzők:Gevers, Tom J. G. Belias, Michail Mustafajev, Ilyas F. Vrolijk, Jan Maarten van Hoek, Bart Bouma, Gerd van Nieuwkerk, Carin M. J. Hartl, Johannes Schramm, Christoph Lohse, Ansgar W. Taubert, Richard Jaeckel, Elmar Manns, Michael P. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Stickel, Felix Heneghan, Michael A. Drenth, Joost P. H.
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5.

001-es BibID:BIBFORM121373
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. - "Accepted by Publisher" (2024). -
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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6.

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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7.

001-es BibID:BIBFORM109814
035-os BibID:(cikkazonosító)193 (scopus)85150952445 (wos)000959451600002
Első szerző:Uhlenbusch, Natalie
Cím:Improving quality of life in patients with rare autoimmune liver diseases by structured peer-delivered support (Q.RARE.LI) : study protocol for a transnational effectiveness-implementation hybrid trial / Natalie Uhlenbusch, Arpinder Bal, Boglárka Balogh, Annika Braun, Anja Geerts, Gideon Hirschfield, Maciej K. Janik, Ansgar W. Lohse, Piotr Milkiewicz, Mária Papp, Carine Poppe, Christoph Schramm, Löwe Bernd
Dátum:2023
ISSN:1471-244X
Megjegyzések:Aims: Psychosocial support is a crucial component of adequate rare disease care, but to date psychosocial support needs of this patient population are insufficiently met. We strive to evaluate the effectiveness of a peer-delivered psychosocial support intervention in patients with rare autoimmune liver diseases in five countries and prepare its implementation. Methods: The psychosocial support program combines structured, manual-based self-help and peer-support. Participant complete a self-help book from home over the course of six weeks, which is based on Acceptance and Commitment Therapy. In addition, they receive weekly telephone-based support sessions with a trained and supervised peer-counsellor. In a first efficacy trial (Depping et al., 2021, JAMA Psychiatry), the program led to improved quality of life, disease acceptance, self-management abilities, social support and reduced helplessness. Within Q.RARE.LI, we will conduct an effectiveness-implementation hybrid trial. To assess effectiveness, we will include N=240 patients with rare autoimmune liver diseases into a two-armed RCT, comparing the support program in addition to care-as-usual (CAU) with CAU alone. Our primary effectiveness outcome is mental health-related quality of life. To assess implementability, we will conduct a mixed-methods process evaluation, examining perceived acceptability and feasibility of the program and explore its implementability with patients, peer-counselors, and healthcare providers. Based on these results, we will derive country-specific implementation strategies. Discussion: The trans-diagnostic and location-independent program has the potential to address the unmet psychosocial support needs of patients with rare diseases. By preparing the implementation in five countries, we hope to improve comprehensive healthcare for an often neglected group.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:BMC Psychiatry. - 23 : 1 (2023), p. 1-11. -
További szerzők:Bal, Arpinder Balogh Boglárka (1993-) (belgyógyász) Braun, Annika Geerts, Anja Hirschfield, Gideon M. Janik, Maciej K. Lohse, Ansgar W. Milkiewicz, Piotr Papp Mária (1975-) (belgyógyász, gasztroenterológus) Poppe, Carine Schramm, Christoph Bernd, Löwe
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8.

001-es BibID:BIBFORM120681
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. - "Accepted by Publisher" (2024). -
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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