High affinity anti‐BSEP antibodies after liver transplantation for PFIC‐2 – Successful treatment with immunoadsorption and B‐cell depletion

摘要: PFIC due to BSEP mutations (PFIC type 2) often necessitates OLT. It has recently been recognized that some PFIC-2 patients develop phenotypic disease recurrence post-OLT the appearance of anti-BSEP antibodies. Here, we describe a boy who became cholestatic four yr after OLT during modification immunosuppression. Canalicular antibody deposits were detected in biopsies transplant and antibodies specifically reacting with identified at high titers his serum. These bound extracellular epitopes inhibited BS transport assumed cause recurrence. Consequently, depletion was pursued by IA B-cell anti-CD20 (rituximab) along switch This treatment resulted prolonged relief symptoms. Depletion pathogenic causing AIBD should be considered as central therapeutic goal.