Post-transplant alloimmune liver disease in bile salt export pump deficiency: A multicenter study.

Authors

Antoine Gardin, Chiara Rubino, Alice Michaut, Géraldine Résin, Marion Almes, Teresa Antonini, Patrick Borentain, Dominique Debray, Eleonora De Martin, Bogdan Hermeziu, Bertrand Roquelaure, Mathias Ruiz, Xavier Stéphenne, Martine Lapalus, Giuseppe Maggiore, Thomas Falguières, Charlotte Mussini, Emmanuel Gonzales, Emmanuel Jacquemin

Published in

JHEP reports : innovation in hepatology. Pages 101969. Jul 25, 2026. Epub Jul 25, 2026.

Abstract

Patients with severe bile salt export pump (BSEP) deficiency may develop alloimmune anti-BSEP liver disease (AIBD) after liver transplantation (LT) due to anti-BSEP antibodies, but its frequency and risk factors are not precisely known.
A multicentre cohort of 35 patients who underwent LT for severe BSEP deficiency was screened retrospectively or prospectively after 2010 for anti-BSEP antibodies. Risk factors, management and outcome of AIBD were analysed.
Ten patients (29%) were screened positive for anti-BSEP antibodies and all developed AIBD, in median 5 years (range: 1.5-14) after LT. Clinical remission of AIBD was achieved in nine patients using various immunosuppressive therapies, with decreased or negative anti-BSEP antibody titers in six patients, all having received rituximab or plasmapheresis/immunoadsorption. Relapse occurred in six patients out of nine, although maintenance therapy using rituximab and/or immunoglobulins enabled prolonged relapse-free survival. Because of AIBD, six patients (60%) required liver retransplantation either at initial AIBD episode or at relapse, and three patients (30%) died. Among the 25 patients without AIBD, one died (4%) and another one was retransplanted (4%). While a severe ABCB11 genotype (biallelic truncating variants) was significantly associated with AIBD (80% in patients with AIBD vs 28% in patients without AIBD, p=0.008), negative BSEP immunostaining on native liver, post-LT CMV infection, presence of biliary anastomosis strictures or graft rejection were not.
In patients with severe BSEP deficiency, AIBD is a frequent post-LT complication that should be screened for, especially in patients with severe ABCB11 genotypes. Early diagnosis and the use of rituximab and plasmapheresis/immunoadsorption may improve patient outcome.
Alloimmune anti-BSEP liver disease is a frequent and severe complication after liver transplantation in patients with BSEP (Bile Salt Export Pump) deficiency, affecting nearly one-third of recipients in this multicentre cohort study. Patients carrying biallelic protein-truncating ABCB11 variants are at particularly high risk and should undergo systematic post-transplant screening for anti-BSEP antibodies. Early recognition of alloimmune anti-BSEP liver disease and timely treatment with B-cell-depleting and antibody-removal therapies, including rituximab and plasmapheresis/ immunoadsorption, may improve outcomes and reduce graft loss. These findings support the implementation of structured surveillance strategies and risk-adapted management in this rare but life-threatening condition.

PMID:
42501969
Bibliographic data and abstract were imported from PubMed on 26 Jul 2026.

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