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

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...

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Veröffentlicht in:Liver international Jg. 44; H. 10; S. 2687 - 2699
Hauptverfasser: 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
Format: Journal Article
Sprache:Englisch
Veröffentlicht: United States Wiley Subscription Services, Inc 01.10.2024
Schlagworte:
Adult
Aged
Alanine
Alanine transaminase
Alanine Transaminase - blood
autoimmune hepatitis
complete biochemical response
Corticoids
Corticosteroids
Diagnosis
Europe
Female
Fibrosis
Hepatitis
Hepatitis, Autoimmune - blood
Hepatitis, Autoimmune - diagnosis
Hepatitis, Autoimmune - drug therapy
Humans
IgG antibody
Immunoglobulin G
Immunoglobulin G - blood
immunosuppression
Immunosuppressive Agents - therapeutic use
Liver
Liver Cirrhosis - blood
Liver Cirrhosis - diagnosis
Liver diseases
Logistic Models
Male
Middle Aged
Multivariate Analysis
Prospective Studies
Rare diseases
Registries
remission
Serum levels
Treatment Outcome
treatment regime
Young Adult
Europe
immunosuppression
treatment regime
autoimmune hepatitis
complete biochemical response
remission
ISSN:1478-3223, 1478-3231, 1478-3231
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Zusammenfassung: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.
Bibliographie:Handling Editor
Alessio Aghemo.
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SourceType-Scholarly Journals-1
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ISSN:1478-3223
1478-3231
1478-3231
DOI:10.1111/liv.16035