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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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2.

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 Htun 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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3.

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 Htun 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
Internet cím:Intézményi repozitóriumban (DEA) tárolt változat
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