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1.
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
Internet cím:
Intézményi repozitóriumban (DEA) tárolt változat
DOI
Borító:
Saját polcon:
2.
001-es BibID:
BIBFORM126381
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 JALM 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 TTL Tjwa, Luise Aamann, Henriette Ytting, Vincenzo Ronca, Kathryn 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 PH 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. - "Accepted by Publisher" (2025). -
További szerzők:
Janik, Maciej K.
Mund, Meike
Uhlenbush, Natalie
Raszeja-Wyszomirska, Joanna
Gerussi, Alessio
Bolis, Francesca
Cristoferi, Laura
Invernizzi, Pietro
Kováts Patrícia (1995-)
Papp Mária (1975-) (belgyógyász, gasztroenterológus)
Grnbk, 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.
Internet cím:
Intézményi repozitóriumban (DEA) tárolt változat
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