Unmet needs in autoimmune hepatitis: Results of the prospective multicentre European Reference Network Registry (R-LIVER)

Ida Schregel; Maria Papp; Nora Sipeki; Patricia J Kovats; Richard Taubert; Bastian Engel; Alejandro Campos-Murguia; George N Dalekos; Nikolaos Gatselis; Kalliopi Zachou; Piotr Milkiewicz; Maciej K Janik; Joanna Raszeja-Wyszomirska; Henriette Ytting; Felix Braun; Christian Casar; Marcial Sebode; Ansgar W Lohse; Christoph Schramm; European Reference Network (ERN) RARE‐LIVER

doi:10.1111/liv.16035

Unmet needs in autoimmune hepatitis: Results of the prospective multicentre European Reference Network Registry (R-LIVER)

Ida Schregel, Maria Papp, Nora Sipeki, Patricia J Kovats, Richard Taubert, Bastian Engel, Alejandro Campos-Murguia, George N Dalekos, Nikolaos Gatselis, Kalliopi Zachou, Piotr Milkiewicz, Maciej K Janik, Joanna Raszeja-Wyszomirska, Henriette Ytting, Felix Braun, Christian Casar, Marcial Sebode, Ansgar W Lohse, Christoph Schramm^*, European Reference Network (ERN) RARE‐LIVER

^*Corresponding author for this work

Gastrounit, Medical Division

17 Citations (Scopus)

Abstract

BACKGROUND AND AIMS: 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 1 year of treatment.

METHODS: Data were 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: A total of 231 patients from six European centres were included in the analysis. After 6 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 6 months. In multivariate analysis, younger age at diagnosis (odds ratio [OR] = 1.03 [95% confidence interval (CI) 1.01-1.05]; p = .007), severe fibrosis (OR .38 [95% .16-.89], p = .026) and change of treatment within the first 6 months (OR .40 [95% CI .2-.86]; p = .018) were associated with a lesser chance of ALT normalization at 12 months follow-up.

CONCLUSION: The landscape of AIH treatment in Europe is highly heterogeneous, 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.

Original language	English
Journal	Liver international : official journal of the International Association for the Study of the Liver
Volume	44
Issue number	10
Pages (from-to)	2687-2699
Number of pages	13
ISSN	1478-3223
DOIs	https://doi.org/10.1111/liv.16035
Publication status	Published - Oct 2024

Keywords

Adult
Aged
Alanine Transaminase/blood
Europe
Female
Hepatitis, Autoimmune/drug therapy
Humans
Immunoglobulin G/blood
Immunosuppressive Agents/therapeutic use
Liver Cirrhosis/diagnosis
Logistic Models
Male
Middle Aged
Multivariate Analysis
Prospective Studies
Registries
Treatment Outcome
Young Adult
immunosuppression
autoimmune hepatitis
complete biochemical response
remission
treatment regime

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10.1111/liv.16035Licence: CC BY-NC-ND

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Schregel, I., Papp, M., Sipeki, N., Kovats, P. J., Taubert, R., Engel, B., Campos-Murguia, A., Dalekos, G. N., Gatselis, N., Zachou, K., Milkiewicz, P., Janik, M. K., Raszeja-Wyszomirska, J., Ytting, H., Braun, F., Casar, C., Sebode, M., Lohse, A. W., Schramm, C., & European Reference Network (ERN) RARE‐LIVER (2024). Unmet needs in autoimmune hepatitis: Results of the prospective multicentre European Reference Network Registry (R-LIVER). Liver international : official journal of the International Association for the Study of the Liver, 44(10), 2687-2699. https://doi.org/10.1111/liv.16035

Unmet needs in autoimmune hepatitis: Results of the prospective multicentre European Reference Network Registry (R-LIVER). / Schregel, Ida; Papp, Maria; Sipeki, Nora et al.
In: Liver international : official journal of the International Association for the Study of the Liver, Vol. 44, No. 10, 10.2024, p. 2687-2699.

Research output: Contribution to journal › Journal article › Research › peer-review

Schregel, I, Papp, M, Sipeki, N, Kovats, PJ, Taubert, R, Engel, B, Campos-Murguia, A, Dalekos, GN, Gatselis, N, Zachou, K, Milkiewicz, P, Janik, MK, Raszeja-Wyszomirska, J, Ytting, H, Braun, F, Casar, C, Sebode, M, Lohse, AW, Schramm, C & European Reference Network (ERN) RARE‐LIVER 2024, 'Unmet needs in autoimmune hepatitis: Results of the prospective multicentre European Reference Network Registry (R-LIVER)', Liver international : official journal of the International Association for the Study of the Liver, vol. 44, no. 10, pp. 2687-2699. https://doi.org/10.1111/liv.16035

@article{b60e5ed0ab634eaba1cd3552f1b8831e,

title = "Unmet needs in autoimmune hepatitis: Results of the prospective multicentre European Reference Network Registry (R-LIVER)",

abstract = "BACKGROUND AND AIMS: 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 1 year of treatment.METHODS: Data were 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: A total of 231 patients from six European centres were included in the analysis. After 6 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 6 months. In multivariate analysis, younger age at diagnosis (odds ratio [OR] = 1.03 [95\% confidence interval (CI) 1.01-1.05]; p = .007), severe fibrosis (OR .38 [95\% .16-.89], p = .026) and change of treatment within the first 6 months (OR .40 [95\% CI .2-.86]; p = .018) were associated with a lesser chance of ALT normalization at 12 months follow-up.CONCLUSION: The landscape of AIH treatment in Europe is highly heterogeneous, 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.",

keywords = "Adult, Aged, Alanine Transaminase/blood, Europe, Female, Hepatitis, Autoimmune/drug therapy, Humans, Immunoglobulin G/blood, Immunosuppressive Agents/therapeutic use, Liver Cirrhosis/diagnosis, Logistic Models, Male, Middle Aged, Multivariate Analysis, Prospective Studies, Registries, Treatment Outcome, Young Adult, immunosuppression, autoimmune hepatitis, complete biochemical response, remission, treatment regime",

author = "Ida Schregel and Maria Papp and Nora Sipeki and Kovats, \{Patricia J\} and Richard Taubert and Bastian Engel and Alejandro Campos-Murguia and Dalekos, \{George N\} and Nikolaos Gatselis and Kalliopi Zachou and Piotr Milkiewicz and Janik, \{Maciej K\} and Joanna Raszeja-Wyszomirska and Henriette Ytting and Felix Braun and Christian Casar and Marcial Sebode and Lohse, \{Ansgar W\} and Christoph Schramm and \{European Reference Network (ERN) RARE‐LIVER\}",

note = "{\textcopyright} 2024 The Author(s). Liver International published by John Wiley \& Sons Ltd.",

year = "2024",

month = oct,

doi = "10.1111/liv.16035",

language = "English",

volume = "44",

pages = "2687--2699",

journal = "Liver international : official journal of the International Association for the Study of the Liver",

issn = "1478-3223",

publisher = "Wiley-Blackwell",

number = "10",

}

TY - JOUR

T1 - Unmet needs in autoimmune hepatitis

T2 - Results of the prospective multicentre European Reference Network Registry (R-LIVER)

AU - Schregel, Ida

AU - Papp, Maria

AU - Sipeki, Nora

AU - Kovats, Patricia J

AU - Taubert, Richard

AU - Engel, Bastian

AU - Campos-Murguia, Alejandro

AU - Dalekos, George N

AU - Gatselis, Nikolaos

AU - Zachou, Kalliopi

AU - Milkiewicz, Piotr

AU - Janik, Maciej K

AU - Raszeja-Wyszomirska, Joanna

AU - Ytting, Henriette

AU - Braun, Felix

AU - Casar, Christian

AU - Sebode, Marcial

AU - Lohse, Ansgar W

AU - Schramm, Christoph

AU - European Reference Network (ERN) RARE‐LIVER

PY - 2024/10

Y1 - 2024/10

N2 - BACKGROUND AND AIMS: 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 1 year of treatment.METHODS: Data were 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: A total of 231 patients from six European centres were included in the analysis. After 6 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 6 months. In multivariate analysis, younger age at diagnosis (odds ratio [OR] = 1.03 [95% confidence interval (CI) 1.01-1.05]; p = .007), severe fibrosis (OR .38 [95% .16-.89], p = .026) and change of treatment within the first 6 months (OR .40 [95% CI .2-.86]; p = .018) were associated with a lesser chance of ALT normalization at 12 months follow-up.CONCLUSION: The landscape of AIH treatment in Europe is highly heterogeneous, 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.

AB - BACKGROUND AND AIMS: 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 1 year of treatment.METHODS: Data were 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: A total of 231 patients from six European centres were included in the analysis. After 6 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 6 months. In multivariate analysis, younger age at diagnosis (odds ratio [OR] = 1.03 [95% confidence interval (CI) 1.01-1.05]; p = .007), severe fibrosis (OR .38 [95% .16-.89], p = .026) and change of treatment within the first 6 months (OR .40 [95% CI .2-.86]; p = .018) were associated with a lesser chance of ALT normalization at 12 months follow-up.CONCLUSION: The landscape of AIH treatment in Europe is highly heterogeneous, 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.

KW - Adult

KW - Aged

KW - Alanine Transaminase/blood

KW - Europe

KW - Female

KW - Hepatitis, Autoimmune/drug therapy

KW - Humans

KW - Immunoglobulin G/blood

KW - Immunosuppressive Agents/therapeutic use

KW - Liver Cirrhosis/diagnosis

KW - Logistic Models

KW - Male

KW - Middle Aged

KW - Multivariate Analysis

KW - Prospective Studies

KW - Registries

KW - Treatment Outcome

KW - Young Adult

KW - immunosuppression

KW - autoimmune hepatitis

KW - complete biochemical response

KW - remission

KW - treatment regime

UR - https://www.scopus.com/pages/publications/85199139220

U2 - 10.1111/liv.16035

DO - 10.1111/liv.16035

M3 - Journal article

C2 - 39037185

SN - 1478-3223

VL - 44

SP - 2687

EP - 2699

JO - Liver international : official journal of the International Association for the Study of the Liver

JF - Liver international : official journal of the International Association for the Study of the Liver

IS - 10

ER -

Unmet needs in autoimmune hepatitis: Results of the prospective multicentre European Reference Network Registry (R-LIVER)

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Keywords

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