Plasma-cell rich rejection and de novo autoimmune hepatitis in pediatric and adult patients -a pre-survey

Aldrian, D;Alrabadi, L;Alterio, T;D´antiga, L;Vogel, G;et.al.
(2026) 58th Annual meeting of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) — Location: Lille, France (24.June.2026)

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Authors
  • Aldrian, D
    Author
  • Alrabadi, L
    Author
  • Alterio, T
    Author
  • D´antiga, L
    Author
  • Jannone, GiuliaUCLouvain
    Author
  • Vogel, G
    Author
  • et. al.
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Abstract
Objectives and Study During the last decades, plasma cell-rich rejection (PCRR), formally de novo autoimmune hepatitis (dnAIH), has been recognized as a cause of late graft dysfunction affecting patients transplanted for hepatic disorders other than autoimmune hepatitis. To date, diagnostic distinction is difficult, treatment recommendations remain inconsistent, and the outcome is unclear. Methods A structured pre-survey questionnaire was distributed via ERN Rare Liver to liver pediatric and adult transplant specialists. The survey covered professional background, nomenclature used at the center, preferred induction therapy, second- and third-line therapy, maintenance therapy strategies, and treatment response. Results A total of 33 collaborators from 30 centers participated, reporting on 293 patients with dnAIH or PCRR (227 pediatric, 66 adult). Prednisolone was the most frequent induction therapy (90.9%, 30/33), followed by increase of prior immunosuppression (18.2%, 6/33), MMF (15.2%, 5/33), other non-specified therapies (12.1%, 4/33), and TNF-α antagonists (3.0%, 1/33). Second-line treatments included MMF (60.6%, 20/33), high-dose prednisone (33.3%, 11/33), and TNF-α antagonists (6.1%, 2/33). For maintenance therapy, azathioprine was most frequently used (66.7%, 22/33), followed by MMF (39.4%, 13/33), low-dose prednisone (36.4%, 12/33), and increase of prior immunosuppression ? which- is it Tac(33.3%, 11/33). First-line therapy achieved sustained remission in 45.5% of cases (15/33). Others noted effective induction but ? relapse (24.2%, 8/33), poor responses (6.1%, 2/33), or claimed insufficient experience (24.2%, 8/33). No significant differences were found between adult and pediatric specialists regarding stable remission (Fisher’s exact test p=0.27) or first-line therapy strategies (p=0.36). Conclusions The pre-survey highlights variability in treatment strategies for PCRR/dnAIH across centers. Although first-line induction therapy is commonly used, second line and maintenance strategies differ widely. These findings underscore the need for standardized definitions and evidence-based protocols to optimize patient outcomes in both pediatric and adult liver transplant recipients. The pre-survey findings will guide a more detailed follow-up analysis of disease features, treatment approaches, and patient outcomes.
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Aldrian, D., Alrabadi, L., Alterio, T., D´antiga, L., de Bruyne, R., Engel, B., Fischler, B., Girard, M., Ranucci, G., Jannone, G., Vogel, G., & et al. (2026). Plasma-cell rich rejection and de novo autoimmune hepatitis in pediatric and adult patients -a pre-survey. 58th Annual meeting of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN), Lille, France. https://hdl.handle.net/2078.5/277828