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, the European Reference Network (ERN) RARE-LIVER
{"title":"自身免疫性肝炎尚未满足的需求:前瞻性多中心欧洲参考网络登记(R-LIVER)的结果。","authors":"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, the European Reference Network (ERN) RARE-LIVER","doi":"10.1111/liv.16035","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Background and Aims</h3>\n \n <p>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.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>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.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>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.</p>\n \n <p>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]; <i>p</i> = .007), severe fibrosis (OR .38 [95% .16–.89], <i>p</i> = .026) and change of treatment within the first 6 months (OR .40 [95% CI .2–.86]; <i>p</i> = .018) were associated with a lesser chance of ALT normalization at 12 months follow-up.</p>\n </section>\n \n <section>\n \n <h3> Conclusion</h3>\n \n <p>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.</p>\n </section>\n </div>","PeriodicalId":18101,"journal":{"name":"Liver International","volume":null,"pages":null},"PeriodicalIF":6.0000,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/liv.16035","citationCount":"0","resultStr":"{\"title\":\"Unmet needs in autoimmune hepatitis: Results of the prospective multicentre European Reference Network Registry (R-LIVER)\",\"authors\":\"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, the European Reference Network (ERN) RARE-LIVER\",\"doi\":\"10.1111/liv.16035\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div>\\n \\n \\n <section>\\n \\n <h3> Background and Aims</h3>\\n \\n <p>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.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Methods</h3>\\n \\n <p>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.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Results</h3>\\n \\n <p>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.</p>\\n \\n <p>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]; <i>p</i> = .007), severe fibrosis (OR .38 [95% .16–.89], <i>p</i> = .026) and change of treatment within the first 6 months (OR .40 [95% CI .2–.86]; <i>p</i> = .018) were associated with a lesser chance of ALT normalization at 12 months follow-up.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Conclusion</h3>\\n \\n <p>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.</p>\\n </section>\\n </div>\",\"PeriodicalId\":18101,\"journal\":{\"name\":\"Liver International\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":6.0000,\"publicationDate\":\"2024-07-22\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://onlinelibrary.wiley.com/doi/epdf/10.1111/liv.16035\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Liver International\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://onlinelibrary.wiley.com/doi/10.1111/liv.16035\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"GASTROENTEROLOGY & HEPATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Liver International","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1111/liv.16035","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
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 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.
期刊介绍:
Liver International promotes all aspects of the science of hepatology from basic research to applied clinical studies. Providing an international forum for the publication of high-quality original research in hepatology, it is an essential resource for everyone working on normal and abnormal structure and function in the liver and its constituent cells, including clinicians and basic scientists involved in the multi-disciplinary field of hepatology. The journal welcomes articles from all fields of hepatology, which may be published as original articles, brief definitive reports, reviews, mini-reviews, images in hepatology and letters to the Editor.