Recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survival

Background & Aims: A significant proportion of patients with variant syndromes (VSs), namely autoimmune hepatitis/primary biliary cholangitis or autoimmune hepatitis/primary sclerosing cholangitis, require liver transplantation (LT) despite treatment. The frequency of disease recurrence and...

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Main Authors: Vincenzo Ronca, Alessandro Parente, Ellina Lytvyak, Bettina E. Hansen, Gideon Hirschfield, Alan Bonder, Maryam Ebadi, Saleh Elwir, Mohamad Alsaed, Piotr Milkiewicz, Maciej K. Janik, Hanns-Ulrich Marschall, Maria Antonella Burza, Cumali Efe, Ali Rıza Calışkan, Murat Harputluoglu, Gökhan Kabaçam, Débora Terrabuio, Fernanda de Quadros Onofrio, Nazia Selzner, Albert Parés, Laura Llovet, Murat Akyıldız, Cigdem Arikan, Mihael P. Manns, Richard Taubert, Anna-Lena Weber, Thomas D. Schiano, Brandy Haydel, Piotr Czubkowski, Piotr Socha, Natalia Ołdak, Nobuhisa Akamatsu, Atsushi Tanaka, Cynthia Levy, Eric F. Martin, Aparna Goel, Mai Sedki, Irena Jankowska, Toru Ikegami, Maria Rodriguez, Martina Sterneck, Marcial Sebode, Christoph Schramm, Maria Francesca Donato, Francesca Colapietro, Ansgar Lohse, Raul J. Andrade, Vilas R. Patwardhan, Bart van Hoek, Maaike Biewenga, Andreas E. Kremer, Yoshihide Ueda, Mark Deneau, Mark Pedersen, Marlyn J. Mayo, Annarosa Floreani, Patrizia Burra, Maria Francesca Secchi, Benedetta Terziroli Beretta-Piccoli, Marco Sciveres, Giuseppe Maggiore, Syed-Mohammed Jafri, Dominique Debray, Muriel Girard, Florence Lacaille, Michael Heneghan, Andrew L. Mason, Ye Oo, Aldo J. Montano-Loza
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Language:English
Published: Elsevier 2025-05-01
Series:JHEP Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2589555925000084
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author Vincenzo Ronca
Alessandro Parente
Ellina Lytvyak
Bettina E. Hansen
Gideon Hirschfield
Alan Bonder
Maryam Ebadi
Saleh Elwir
Mohamad Alsaed
Piotr Milkiewicz
Maciej K. Janik
Hanns-Ulrich Marschall
Maria Antonella Burza
Cumali Efe
Ali Rıza Calışkan
Murat Harputluoglu
Gökhan Kabaçam
Débora Terrabuio
Fernanda de Quadros Onofrio
Nazia Selzner
Albert Parés
Laura Llovet
Murat Akyıldız
Cigdem Arikan
Mihael P. Manns
Richard Taubert
Anna-Lena Weber
Thomas D. Schiano
Brandy Haydel
Piotr Czubkowski
Piotr Socha
Natalia Ołdak
Nobuhisa Akamatsu
Atsushi Tanaka
Cynthia Levy
Eric F. Martin
Aparna Goel
Mai Sedki
Irena Jankowska
Toru Ikegami
Maria Rodriguez
Martina Sterneck
Marcial Sebode
Christoph Schramm
Maria Francesca Donato
Francesca Colapietro
Ansgar Lohse
Raul J. Andrade
Vilas R. Patwardhan
Bart van Hoek
Maaike Biewenga
Andreas E. Kremer
Yoshihide Ueda
Mark Deneau
Mark Pedersen
Marlyn J. Mayo
Annarosa Floreani
Patrizia Burra
Maria Francesca Secchi
Benedetta Terziroli Beretta-Piccoli
Marco Sciveres
Giuseppe Maggiore
Syed-Mohammed Jafri
Dominique Debray
Muriel Girard
Florence Lacaille
Michael Heneghan
Andrew L. Mason
Ye Oo
Aldo J. Montano-Loza
author_facet Vincenzo Ronca
Alessandro Parente
Ellina Lytvyak
Bettina E. Hansen
Gideon Hirschfield
Alan Bonder
Maryam Ebadi
Saleh Elwir
Mohamad Alsaed
Piotr Milkiewicz
Maciej K. Janik
Hanns-Ulrich Marschall
Maria Antonella Burza
Cumali Efe
Ali Rıza Calışkan
Murat Harputluoglu
Gökhan Kabaçam
Débora Terrabuio
Fernanda de Quadros Onofrio
Nazia Selzner
Albert Parés
Laura Llovet
Murat Akyıldız
Cigdem Arikan
Mihael P. Manns
Richard Taubert
Anna-Lena Weber
Thomas D. Schiano
Brandy Haydel
Piotr Czubkowski
Piotr Socha
Natalia Ołdak
Nobuhisa Akamatsu
Atsushi Tanaka
Cynthia Levy
Eric F. Martin
Aparna Goel
Mai Sedki
Irena Jankowska
Toru Ikegami
Maria Rodriguez
Martina Sterneck
Marcial Sebode
Christoph Schramm
Maria Francesca Donato
Francesca Colapietro
Ansgar Lohse
Raul J. Andrade
Vilas R. Patwardhan
Bart van Hoek
Maaike Biewenga
Andreas E. Kremer
Yoshihide Ueda
Mark Deneau
Mark Pedersen
Marlyn J. Mayo
Annarosa Floreani
Patrizia Burra
Maria Francesca Secchi
Benedetta Terziroli Beretta-Piccoli
Marco Sciveres
Giuseppe Maggiore
Syed-Mohammed Jafri
Dominique Debray
Muriel Girard
Florence Lacaille
Michael Heneghan
Andrew L. Mason
Ye Oo
Aldo J. Montano-Loza
author_sort Vincenzo Ronca
collection DOAJ
description Background &amp; Aims: A significant proportion of patients with variant syndromes (VSs), namely autoimmune hepatitis/primary biliary cholangitis or autoimmune hepatitis/primary sclerosing cholangitis, require liver transplantation (LT) despite treatment. The frequency of disease recurrence and the effect on graft survival are yet to be clarified. The aim of this international, multicentric, retrospective study is to evaluate the risk factors associated with recurrence and the impact of the disease recurrence after LT on graft and patient survival. Methods: We evaluated 166 patients undergoing LT for VS in 33 centers in North America, South America, Europe, and Asia. Clinical data before and after LT, biochemical data within the first 12 months after LT, and immunosuppression after LT were analyzed to identify patients with a higher risk of recurrence of autoimmune disease based on a histological and radiological diagnosis. Cumulative probabilities of graft and overall survival after LT were calculated using a semi-Markov model. Results: The autoimmune pattern of recurrence resembled the original VS in 19 cases (61%). Recurrence of autoimmune liver disease (rALD) after LT was observed in 23% and 33% of patients after 5 and 10 years, respectively. Increased alkaline phosphatase (hazard ratio [HR] 1.60, 95% confidence interval [CI] 1.13–2.25, p <0.01) and alanine aminotransferase (HR 1.25, 95% CI 1.01–1.53, p = 0.03) at 12 months after LT and acute rejection (HR 3.58, 95% CI 1.60–7.73, p <0.01) were associated with a higher risk of VS recurrence, whereas the use of predniso(lo)ne was associated with a reduced risk (HR 0.30, 95% CI 0.14–0.64, p <0.01). After adjusting for alanine aminotransferase and alkaline phosphatase at 12 months, the use of predniso(lo)ne was found to be independently and negatively associated with recurrent disease. The rALD was found to be significantly associated with graft loss and patient survival in the multivariate Cox regression analysis with a time-dependent covariate. The 5- and 10-year probabilities of graft survival were 68% and 41% in patients with recurrent VS compared with 83% and 60% in patients without recurrent disease, respectively (p = 0.01). The overall survival was significantly reduced in patients with recurrent disease (p = 0.01), with event probability at 5 and 10 years of 75% and 49% vs. 84% and 60% in patients without recurrence, respectively. Conclusions: rALD after LT is frequent and is associated with elevation in liver enzymes within the first year after LT and rejection episodes. According to our data, VS recurrence appears to be associated with poorer graft and patient survival. Further studies are needed to explore strategies that can prevent VS recurrence or mitigate its potential impact. Impact and implications: This study investigated the recurrence of autoimmune liver diseases (rALD) in patients transplanted for variant syndromes (VSs) and its effect on graft and patient survival. The findings reveal a significant association between rALD and poorer graft and overall survival, highlighting the need for preventive strategies. This research is crucial for transplant physicians and healthcare providers, as it underscores the impact of early liver enzyme monitoring and tailored immunosuppressive therapy on long-term outcomes. These insights can inform more effective post-LT management protocols, potentially improving patient prognosis.
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spelling doaj-art-687b8c158bef48d6afc4c659e1e8e7f12025-08-20T03:53:01ZengElsevierJHEP Reports2589-55592025-05-017510133210.1016/j.jhepr.2025.101332Recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survivalVincenzo Ronca0Alessandro Parente1Ellina Lytvyak2Bettina E. Hansen3Gideon Hirschfield4Alan Bonder5Maryam Ebadi6Saleh Elwir7Mohamad Alsaed8Piotr Milkiewicz9Maciej K. Janik10Hanns-Ulrich Marschall11Maria Antonella Burza12Cumali Efe13Ali Rıza Calışkan14Murat Harputluoglu15Gökhan Kabaçam16Débora Terrabuio17Fernanda de Quadros Onofrio18Nazia Selzner19Albert Parés20Laura Llovet21Murat Akyıldız22Cigdem Arikan23Mihael P. Manns24Richard Taubert25Anna-Lena Weber26Thomas D. Schiano27Brandy Haydel28Piotr Czubkowski29Piotr Socha30Natalia Ołdak31Nobuhisa Akamatsu32Atsushi Tanaka33Cynthia Levy34Eric F. Martin35Aparna Goel36Mai Sedki37Irena Jankowska38Toru Ikegami39Maria Rodriguez40Martina Sterneck41Marcial Sebode42Christoph Schramm43Maria Francesca Donato44Francesca Colapietro45Ansgar Lohse46Raul J. Andrade47Vilas R. Patwardhan48Bart van Hoek49Maaike Biewenga50Andreas E. Kremer51Yoshihide Ueda52Mark Deneau53Mark Pedersen54Marlyn J. Mayo55Annarosa Floreani56Patrizia Burra57Maria Francesca Secchi58Benedetta Terziroli Beretta-Piccoli59Marco Sciveres60Giuseppe Maggiore61Syed-Mohammed Jafri62Dominique Debray63Muriel Girard64Florence Lacaille65Michael Heneghan66Andrew L. Mason67Ye Oo68Aldo J. Montano-Loza69Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; IRCCS Humanitas Research Hospital, Rozzano, Italy; Corresponding authors. Addresses: Division of Internal Medicine and Hepatology, Humanitas Research Hospital, European Reference Network (ERN) RARE-LIVER Centre (www.rare-liver.eu), Via A. Manzoni 56, 20089, Rozzano, MI, Italy. Tel.: 0282243787 (V. Ronca); Division of Gastroenterology and Liver Unit, 8540 112 Street NW, Zeidler Ledcor Center, University of Alberta, Edmonton, AB, T6G 2X8, Canada. Tel.: (780) 248-1892; Fax: (780) 248-1895 (A. Montano-Loza); Centre for Liver and Gastro Research, University of Birmingham, Liver Transplant and Hepatobiliary Unit, Centre for Rare Disease and ERN Rare Liver Centre, UHB NHS Foundation Trust, London, UK (Y.H. Oo).Institute of Liver Studies, King’s College Hospital NHS Foundation Trust, Denmark Hill, SE59RS, London, UKDepartment of Medicine, University of Alberta, Edmonton, AB, CanadaDepartment of Epidemiology and Biostatistics, Erasmus MC, Rotterdam, The Netherlands; European Reference Network for Hepatological Diseases (ERN RARE-LIVER)Toronto Center for Liver Disease, University Health Network, University of Toronto, Toronto, ON, CanadaBeth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USADivision of Gastroenterology and Liver Unit, University of Alberta, Edmonton, AB, CanadaBaylor University Medical Center, Dallas, TX, USABaylor University Medical Center, Dallas, TX, USAEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Liver and Internal Medicine Unit, Medical University of Warsaw, Warsaw, PolandEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Liver and Internal Medicine Unit, Medical University of Warsaw, Warsaw, PolandEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Sahlgrenska University Hospital, Gothenburg, SwedenEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Gastroenterology and Hepatology, Sahlgrenska University Hospital, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE LIVER), Gothenburg, SwedenDepartment of Gastroenterology, Harran University Hospital, Şanlıurfa, TurkeyDepartment of Gastroenterology, Adiyaman University School of Medicine, Adiyaman, TurkeyDepartment of Gastroenterology, Inönü University School of Medicine, Malatya, TurkeyClinic of Gastroenterology and Liver Transplantation, Guven Hospital, Ankara, TurkeyDepartment of Gastroenterology, University of São Paulo School of Medicine, São Paulo, BrazilToronto Center for Liver Disease, University Health Network, University of Toronto, Toronto, ON, CanadaToronto Center for Liver Disease, University Health Network, University of Toronto, Toronto, ON, CanadaEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Liver Unit, Hospital Clínic, University of Barcelona, IDIBAPS, CIBERehd, Barcelona SpainEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Liver Unit, Hospital Clínic, University of Barcelona, IDIBAPS, CIBERehd, Barcelona SpainKoç University School of Medicine, Department of Gastroenterology and Liver Transplantation Center, Istanbul, TurkeyKoc University School of Medicine Pediatric Gastroenterology and Hepatology, Organ Transplantation Center, Koc University Research Center for Translational Medicine (KUTTAM), Istanbul, TurkeyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, GermanyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, GermanyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, GermanyRecanati/Miller Transplantation Institute/Division of Liver Diseases, Mount Sinai Medical Center, New York, NY, USARecanati/Miller Transplantation Institute/Division of Liver Diseases, Mount Sinai Medical Center, New York, NY, USAEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, PolandEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, PolandEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, PolandHepato-Biliary-Pancreatic Surgery Division, Artificial Organ and Transplantation Division, Department of Surgery, The University of Tokyo, Tokyo, JapanDepartment of Medicine, Teikyo University School of Medicine, Tokyo, JapanDepartment of Medicine, Division of Digestive Health and Liver Diseases, University of Miami Miller School of Medicine, Miami, Florida, USADepartment of Medicine, Division of Digestive Health and Liver Diseases, University of Miami Miller School of Medicine, Miami, Florida, USADivision of Gastroenterology &amp; Hepatology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USADepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanHepato-Biliary-Pancreatic Surgery Division, Artificial Organ and Transplantation Division, Department of Surgery, The University of Tokyo, Tokyo, JapanDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); UKE Hamburg, Hamburg, GermanyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); UKE Hamburg, Hamburg, GermanyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); UKE Hamburg, Hamburg, GermanyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); UKE Hamburg, Hamburg, GermanyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Liver Transplant Hepatology Unit, Division of Gastroenterology and Hepatology, Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy; IRCCS Humanitas Research Hospital, Rozzano, ItalyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); UKE Hamburg, Hamburg, GermanyGastroenterology Service—IBIMA, University Hospital and CIBERehd, University of Málaga, Málaga, SpainBeth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USALeiden University Medical Center, Leiden, The NetherlandsLeiden University Medical Center, Leiden, The NetherlandsEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Medicine, University Hospital Erlangen and Friedrich-Alexander-University Erlangen-Nürnberg, Erlangen, Germany; Department of Gastroenterology and Hepatology, University Hospital Zürich, University of Zürich, Zürich, SwitzerlandDepartment of Gastroenterology and Hepatology, Graduate School of Medicine, Kyoto University, Kyoto, JapanUniversity of Utah and Intermountain Healthcare Primary Children's Hospital, Salt Lake City, Utah, USAThe University of Texas Southwestern Medical Center, Dallas, TX, USAThe University of Texas Southwestern Medical Center, Dallas, TX, USAEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Surgery, Oncology and Gastroenterology, University of Padova, Padova, ItalyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Department of Surgery, Oncology and Gastroenterology, University of Padova, Padova, ItalyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); University of Padova, Padova, ItalyEpatocentro Ticino &amp; Università della Svizzera Italiana, Lugano, SwitzerlandUPMC Pediatric Liver Center, Palermo, ItalyEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Hepatogastroenterology, Nutrition and Liver Transplant IRCCS Bambino Gesù Pediatric Hospital, Rome ItalyHenry Ford Health System, Michigan, USAEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Pediatric Liver Unit, Paris Descartes University and French National Reference Center for Rare Diseases BA and Genetic Cholestasis, Hôpital Necker, Paris, FranceEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Pediatric Liver Unit, Paris Descartes University and French National Reference Center for Rare Diseases BA and Genetic Cholestasis, Hôpital Necker, Paris, FranceEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Pediatric Liver Unit, Paris Descartes University and French National Reference Center for Rare Diseases BA and Genetic Cholestasis, Hôpital Necker, Paris, FranceInstitute of Liver Studies, King’s College Hospital NHS Foundation Trust, Denmark Hill, SE59RS, London, UKDivision of Gastroenterology and Liver Unit, University of Alberta, Edmonton, AB, CanadaEuropean Reference Network for Hepatological Diseases (ERN RARE-LIVER); Centre for Liver and Gastro Research, Birmingham NIHR Inflammation Biomedical Research, Birmingham, UK; Centre Liver Unit, Queen Elizabeth University Hospital Birmingham, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK; Corresponding authors. Addresses: Division of Internal Medicine and Hepatology, Humanitas Research Hospital, European Reference Network (ERN) RARE-LIVER Centre (www.rare-liver.eu), Via A. Manzoni 56, 20089, Rozzano, MI, Italy. Tel.: 0282243787 (V. Ronca); Division of Gastroenterology and Liver Unit, 8540 112 Street NW, Zeidler Ledcor Center, University of Alberta, Edmonton, AB, T6G 2X8, Canada. Tel.: (780) 248-1892; Fax: (780) 248-1895 (A. Montano-Loza); Centre for Liver and Gastro Research, University of Birmingham, Liver Transplant and Hepatobiliary Unit, Centre for Rare Disease and ERN Rare Liver Centre, UHB NHS Foundation Trust, London, UK (Y.H. Oo).Division of Gastroenterology and Liver Unit, University of Alberta, Edmonton, AB, Canada; Corresponding authors. Addresses: Division of Internal Medicine and Hepatology, Humanitas Research Hospital, European Reference Network (ERN) RARE-LIVER Centre (www.rare-liver.eu), Via A. Manzoni 56, 20089, Rozzano, MI, Italy. Tel.: 0282243787 (V. Ronca); Division of Gastroenterology and Liver Unit, 8540 112 Street NW, Zeidler Ledcor Center, University of Alberta, Edmonton, AB, T6G 2X8, Canada. Tel.: (780) 248-1892; Fax: (780) 248-1895 (A. Montano-Loza); Centre for Liver and Gastro Research, University of Birmingham, Liver Transplant and Hepatobiliary Unit, Centre for Rare Disease and ERN Rare Liver Centre, UHB NHS Foundation Trust, London, UK (Y.H. Oo).Background &amp; Aims: A significant proportion of patients with variant syndromes (VSs), namely autoimmune hepatitis/primary biliary cholangitis or autoimmune hepatitis/primary sclerosing cholangitis, require liver transplantation (LT) despite treatment. The frequency of disease recurrence and the effect on graft survival are yet to be clarified. The aim of this international, multicentric, retrospective study is to evaluate the risk factors associated with recurrence and the impact of the disease recurrence after LT on graft and patient survival. Methods: We evaluated 166 patients undergoing LT for VS in 33 centers in North America, South America, Europe, and Asia. Clinical data before and after LT, biochemical data within the first 12 months after LT, and immunosuppression after LT were analyzed to identify patients with a higher risk of recurrence of autoimmune disease based on a histological and radiological diagnosis. Cumulative probabilities of graft and overall survival after LT were calculated using a semi-Markov model. Results: The autoimmune pattern of recurrence resembled the original VS in 19 cases (61%). Recurrence of autoimmune liver disease (rALD) after LT was observed in 23% and 33% of patients after 5 and 10 years, respectively. Increased alkaline phosphatase (hazard ratio [HR] 1.60, 95% confidence interval [CI] 1.13–2.25, p <0.01) and alanine aminotransferase (HR 1.25, 95% CI 1.01–1.53, p = 0.03) at 12 months after LT and acute rejection (HR 3.58, 95% CI 1.60–7.73, p <0.01) were associated with a higher risk of VS recurrence, whereas the use of predniso(lo)ne was associated with a reduced risk (HR 0.30, 95% CI 0.14–0.64, p <0.01). After adjusting for alanine aminotransferase and alkaline phosphatase at 12 months, the use of predniso(lo)ne was found to be independently and negatively associated with recurrent disease. The rALD was found to be significantly associated with graft loss and patient survival in the multivariate Cox regression analysis with a time-dependent covariate. The 5- and 10-year probabilities of graft survival were 68% and 41% in patients with recurrent VS compared with 83% and 60% in patients without recurrent disease, respectively (p = 0.01). The overall survival was significantly reduced in patients with recurrent disease (p = 0.01), with event probability at 5 and 10 years of 75% and 49% vs. 84% and 60% in patients without recurrence, respectively. Conclusions: rALD after LT is frequent and is associated with elevation in liver enzymes within the first year after LT and rejection episodes. According to our data, VS recurrence appears to be associated with poorer graft and patient survival. Further studies are needed to explore strategies that can prevent VS recurrence or mitigate its potential impact. Impact and implications: This study investigated the recurrence of autoimmune liver diseases (rALD) in patients transplanted for variant syndromes (VSs) and its effect on graft and patient survival. The findings reveal a significant association between rALD and poorer graft and overall survival, highlighting the need for preventive strategies. This research is crucial for transplant physicians and healthcare providers, as it underscores the impact of early liver enzyme monitoring and tailored immunosuppressive therapy on long-term outcomes. These insights can inform more effective post-LT management protocols, potentially improving patient prognosis.http://www.sciencedirect.com/science/article/pii/S2589555925000084AutoimmunityRecurrenceLiver transplantation
spellingShingle Vincenzo Ronca
Alessandro Parente
Ellina Lytvyak
Bettina E. Hansen
Gideon Hirschfield
Alan Bonder
Maryam Ebadi
Saleh Elwir
Mohamad Alsaed
Piotr Milkiewicz
Maciej K. Janik
Hanns-Ulrich Marschall
Maria Antonella Burza
Cumali Efe
Ali Rıza Calışkan
Murat Harputluoglu
Gökhan Kabaçam
Débora Terrabuio
Fernanda de Quadros Onofrio
Nazia Selzner
Albert Parés
Laura Llovet
Murat Akyıldız
Cigdem Arikan
Mihael P. Manns
Richard Taubert
Anna-Lena Weber
Thomas D. Schiano
Brandy Haydel
Piotr Czubkowski
Piotr Socha
Natalia Ołdak
Nobuhisa Akamatsu
Atsushi Tanaka
Cynthia Levy
Eric F. Martin
Aparna Goel
Mai Sedki
Irena Jankowska
Toru Ikegami
Maria Rodriguez
Martina Sterneck
Marcial Sebode
Christoph Schramm
Maria Francesca Donato
Francesca Colapietro
Ansgar Lohse
Raul J. Andrade
Vilas R. Patwardhan
Bart van Hoek
Maaike Biewenga
Andreas E. Kremer
Yoshihide Ueda
Mark Deneau
Mark Pedersen
Marlyn J. Mayo
Annarosa Floreani
Patrizia Burra
Maria Francesca Secchi
Benedetta Terziroli Beretta-Piccoli
Marco Sciveres
Giuseppe Maggiore
Syed-Mohammed Jafri
Dominique Debray
Muriel Girard
Florence Lacaille
Michael Heneghan
Andrew L. Mason
Ye Oo
Aldo J. Montano-Loza
Recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survival
JHEP Reports
Autoimmunity
Recurrence
Liver transplantation
title Recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survival
title_full Recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survival
title_fullStr Recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survival
title_full_unstemmed Recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survival
title_short Recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survival
title_sort recurrence of autoimmune hepatitis cholestatic variant syndromes after liver transplantation affects graft and patient survival
topic Autoimmunity
Recurrence
Liver transplantation
url http://www.sciencedirect.com/science/article/pii/S2589555925000084
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AT thomasdschiano recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT brandyhaydel recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT piotrczubkowski recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT piotrsocha recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT nataliaołdak recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT nobuhisaakamatsu recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT atsushitanaka recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT cynthialevy recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT ericfmartin recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT aparnagoel recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT maisedki recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT irenajankowska recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT toruikegami recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT mariarodriguez recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT martinasterneck recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT marcialsebode recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT christophschramm recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT mariafrancescadonato recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT francescacolapietro recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT ansgarlohse recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT rauljandrade recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT vilasrpatwardhan recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT bartvanhoek recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT maaikebiewenga recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT andreasekremer recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT yoshihideueda recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT markdeneau recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT markpedersen recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT marlynjmayo recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT annarosafloreani recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT patriziaburra recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT mariafrancescasecchi recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT benedettaterziroliberettapiccoli recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT marcosciveres recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT giuseppemaggiore recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT syedmohammedjafri recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT dominiquedebray recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT murielgirard recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT florencelacaille recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT michaelheneghan recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT andrewlmason recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT yeoo recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival
AT aldojmontanoloza recurrenceofautoimmunehepatitischolestaticvariantsyndromesafterlivertransplantationaffectsgraftandpatientsurvival