Complementary use of autoantibody detection methods facilitates diagnosis of juvenile autoimmune hepatitis and autoimmune sclerosing cholangitis
Autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (AISC) are rare paediatric liver diseases that can be difficult to distinguish from other hepatopathies. Autoantibody testing is central to diagnosis, yet paediatric performance across platforms has been poorly standardised and sparsely reported. In this multicentre comparison (202 sera from nine expert centres across eight European countries), indirect immunofluorescence (IFT) on rodent tissue showed suboptimal discrimination at the commonly advocated 1:20 cut-off, whereas accuracy for ANA and SMA improved markedly at 1:320. ANA detection by IFT on HEp-2 cells and by ELISA was comparable to rodent tissue IFT. ELISAs for F-actin and pIgG achieved the highest AUCs for identifying AIH/AISC and may complement current diagnostics. Substantial inter-platform discordance for ANA underscores the need for harmonisation. Collectively, these data support the use of multiple validated platforms with platform-appropriate cut-offs in paediatric AIH/AISC serology and support updates to diagnostic algorithms to improve diagnostic timeliness and reliability.
Authors
- Simon Pape (ORCID: https://orcid.org/0000-0002-3770-4568)
- Bastian Engel (ORCID: https://orcid.org/0000-0002-0972-3454)
- Ye Htun Oo (ORCID: https://orcid.org/0000-0002-0495-6734)
- Amédée Renand (ORCID: https://orcid.org/0000-0003-2265-549X)
- Claudine Lalanne
- George Ν. Dalekos (ORCID: https://orcid.org/0000-0001-7075-8464)
- Theresa Kirchner (ORCID: https://orcid.org/0000-0003-2094-1505)
- Wojciech Jańczyk (ORCID: https://orcid.org/0000-0002-6430-0788)
- Piotr Socha (ORCID: https://orcid.org/0000-0002-1621-464X)
- Richard Taubert (ORCID: https://orcid.org/0000-0001-9270-2496)
- Jérôme Gournay (ORCID: https://orcid.org/0000-0002-9160-9369)
- Luigi Muratori (ORCID: https://orcid.org/0000-0002-8748-8031)
- Norman Junge (ORCID: https://orcid.org/0000-0002-3099-2667)
- Çiğdem Arıkan (ORCID: https://orcid.org/0000-0002-0794-2741)
- Muhammed Yüksel (ORCID: https://orcid.org/0000-0002-3390-6007)
- Joost P.H. Drenth (ORCID: https://orcid.org/0000-0001-8027-3073)
- Heiner H. Wedemeyer (ORCID: https://orcid.org/0000-0003-2906-0480)
- Kalliopi Zachou (ORCID: https://orcid.org/0000-0002-4218-9847)
- Nicole Henjes
- Yun Ma
- Ulrich Baumann
- Stephanie Loges
Institutions
- Koç University (TR)
- Radboud University Nijmegen (NL)
- Inserm (FR)
- King's College London (GB)
- Radboud University Medical Center (NL)
- Medizinische Hochschule Hannover (DE)
- NIHR Birmingham Liver Biomedical Research Unit (GB)
- University Hospital of Larissa (GR)
- Children's Memorial Health Institute (PL)
- Centre d'Investigation Clinique de Nantes (FR)
- Amsterdam University Medical Centers (NL)
- King's College Hospital (GB)
- NIHR Birmingham Biomedical Research Centre (GB)
- ERN RARE-LIVER (DE)
- University of Bologna (IT)
- Nantes Université (FR)
Publication Details
- Journal
- JHEP Reports
- Published
- 2025-12-06
- DOI
- https://doi.org/10.1016/j.jhepr.2025.101706
- Citations
- 1
- Primary Topic
- Liver Diseases and Immunity
- Type
- article
- Field-Weighted Citation Impact
- 1.32
Funders
- Deutsche Forschungsgemeinschaft
- Medizinischen Hochschule Hannover