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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:	BIBFORM126381
035-os BibID:	(Scopus)85218769201 (WoS)001510104200001
Első szerző:	Snijders, Romee
Cím:	Health-related quality of life is impaired in people with autoimmune hepatitis : results of a multicentre cross sectional study within the European Reference Network / Romée J. A. L. M. Snijders, Maciej K. Janik, Meike Mund, Natalie Uhlenbusch, Joanna Raszeja-Wyszomirska, Alessio Gerussi, Francesca Bolis, Laura Cristoferi, Pietro Invernizzi, Patricia Kovats, Mária Papp, Lisbet Grønbæk, Henning Grønbæk, Eric T. T. L. Tjwa, Luise Aamann, Henriette Ytting, Vincenzo Ronca, Katheryn Olsen, Ye H. Oo, Adriaan J. van der Meer, João Madaleno, Bernardo Canhão, Bastian Engel, Alejandro Campos-Murguia, Richard Taubert, Özgür M-Koc, Matthijs Kramer, José A. Willemse, Bernd Löwe, Ansgar W. Lohse, Joost P. H. Drenth, Christoph Schramm, Piotr Milkiewicz, Tom J. Gevers
Dátum:	2025
ISSN:	0270-9139
Megjegyzések:	Abstract Background: Impaired health-related quality of life (HRQoL) contributes to the overall disease burden in autoimmune hepatitis (AIH). This study aimed to evaluate HRQoL in people with AIH and to identify potentially modifiable factors associated with impaired HRQoL using validated patient-reported outcome measures. Methods: Adult AIH patients diagnosed at 12 European centers were enrolled in this prospective, cross-sectional study from July 2020-June 2023. HRQoL was assessed using the Physical Component Score (PCS) and Mental Component Score (MCS) of the 12-item Short Form Health Survey (SF-12), and the European Quality-of-life 5-Dimension 5-Level (EQ-5D-5L) utility index (UI) score. Mixed-model regression analyses identified factors associated with HRQoL and somatic symptom severity. Controls were recruited from the general population in five European countries. Results: A total of 882 patients with AIH (mean age: 51.0 years [SD 17.0]; 76.4% female) and 178 controls were included. Physical but not mental HRQoL was impaired in the AIH group compared with the control group (PCS: 46.3 vs. 51.9, p<0.001; EQ-5D UI: 0.87 vs. 0.95, p<0.001). HRQoL was associated with severe somatic symptoms (PCS ? = -4.26, p<0.001), fatigue (PCS ? = -0.25, p<0.001; MCS ? = -0.25, p<0.001), and depression/ anxiety (PCS ? = 3.37, p<0.001; MCS ? = -6.79, p<0.001). A complete biochemical responseCBR was associated with a lower somatic symptom severity (odds ratio 0.69, p<0.05). Conclusions: People with AIH had significantly impaired HRQoL compared with controls, particularly in terms of physical well-being. HRQoL scores are associated with symptom burden, encompassing both somatic and psychosocial dimensions.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk mental health physical health depression complete biochemical response symptoms
Megjelenés:	Hepatology. - 82 : 5 (2025), p. 1058-1072. -
További szerzők:	Janik, Maciej K. Mund, Meike Uhlenbusch, Natalie Raszeja-Wyszomirska, Joanna Gerussi, Alessio Bolis, Francesca Cristoferi, Laura Invernizzi, Pietro Kováts Patrícia (1995-) Papp Mária (1975-) (belgyógyász, gasztroenterológus) Groenbaek, Lisbet Groenbaek, Henning Tjwa, Eric Aamann, Luise Ytting, Henriette Ronca, Vincenzo Olsen, Katheryn Oo, Ye H. Meer, Adriaan J. van der Madaleno, João Canhão, Bernardo Engel, Bastian Campos-Murguia, Alejandro Taubert, Richard M-Koc, Özgür Kramer, Matthijs Willemse, José A. Löwe, Bernd Lohse, Ansgar W. Drenth, Joost P. H. Schramm, Christoph Milkiewicz, Piotr Gevers, Tom J. G.
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001-es BibID:	BIBFORM134841
Első szerző:	Uhlenbusch, Natalie
Cím:	Quality of life in patients with variant syndromes of autoimmune liver diseases : a cross-sectional multicenter study / Natalie Uhlenbusch, Romée J. A. L. M. Snijder, Meike Mund, Maciej K. Janik, Piotr Milkiewicz, Bernd Löwe, Claudia Kroll, Joanna Raszeja-Wyszomirska, Alessio Gerussi, Francesca Bolis, Laura Cristoferi, Pietro Invernizzi, Patricia Kovats, Mária Papp, Lisbet Grnbk, Henning Grnbk, Eric T. T. L. Tjwa, Luise Aamann, Henriette Ytting, Vincenzo Ronca, Kathryn Olsen, Ye H. Oo, Adriaan J. van der Meer, Joao Madaleno, Bernardo Canhao, Bastian Engel, Alejandro Campos-Murguia, Richard Taubert, Özgür M. Koc, Matthijs Kramer, José A Willemse, Ansgar W. Lohse, Joost P. H. Drenth, Tom J. G. Gevers, Christoph Schramm
Dátum:	2026
ISSN:	1478-3223
Megjegyzések:	Background & Aims Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) go along with reduced health-related quality of life (HRQOL). Variant syndromes, i.e. conditions with features of both PBC/PSC and AIH, are associated with higher clinical complexity and worse prognosis. Studies on HRQOL in patients with variant syndromes are lacking. We aimed to provide large-scale evidence addressing this gap. Methods We included adult patients with clinical diagnoses of autoimmune liver diseases across nine countries in a cross-sectional study. We descriptively compared demographical, clinical and patient-reported outcomes between the conditions and investigated whether additional AIH contributes to reduced HRQOL compared to the cholestatic liver disease alone. Further, we explored the role of fatigue, cirrhosis and depression severity regarding HRQOL. Results N=1275 patients were included (PBC: n=342, PBC-AIH: n=160, PSC: n=305, PSC-AIH: n=121, AIH: n=347). Patients with variant syndromes showed high rates of cirrhosis and increased depressive or anxiety symptoms. Additional AIH was associated with further reduction in physical and mental HRQOL in people with PSC (physical: ?R2=.012, p=.041; mental: ?R2=.016, p=.020), but not with PBC (physical: ?R2=.008, p=.081; mental: ?R2=.001, p=.609). Physical HRQOL was associated with higher age and fatigue, while mental HRQOL was associated with lower age, fatigue and depression severity. Conclusions Patients with variant syndromes of autoimmune liver diseases show high physical and mental burden, with fatigue as the main contributor. Particularly PSC-AIH goes along with more severely reduced HRQOL compared to the cholestatic liver disease alone, which is attributable to higher symptom burden.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk autoimmune liver diseases cholestatic liver diseases autoimmune hepatitis variant syndrome overlap syndrome quality of life
Megjelenés:	Liver International. - "Accepted by Publisher" (2026). -
További szerzők:	Snijders, Romee Mund, Meike Janik, Maciej K. Milkiewicz, Piotr Löwe, Bernd Kroll, Claudia Raszeja-Wyszomirska, Joanna Gerussi, Alessio Bolis, Francesca Cristoferi, Laura Invernizzi, Pietro Kováts Patrícia (1995-) Papp Mária (1975-) (belgyógyász, gasztroenterológus) Grnbk, Lisbet Grnbk, Henning Tjwa, Eric Aamann, Luise Ytting, Henriette Ronca, Vincenzo Olsen, Katheryn Oo, Ye H. van der Meer, Adriaan J. Madaleno, João Canhão, Bernardo Engel, Bastian Campos-Murguia, Alejandro Taubert, Richard Koc, Özgür M. Kramer, Matthijs Willemse, José A. Lohse, Ansgar W. Drenth, Joost P. H. Gevers, Tom J. G. Schramm, Christoph
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