Iterative antibody-induced bile salt export pump deficiency after successive liver transplantations successfully treated with plasmapheresis and rituximab

Clin Res Hepatol Gastroenterol. 2023 May;47(6):102139. doi: 10.1016/j.clinre.2023.102139. Epub 2023 May 13.

Abstract

Post-transplantation evolution of progressive familial intrahepatic cholestasis type 2 patients can be complicated by antibody-induced bile salt export pump deficiency (AIBD). There is no consensus on its management. We describe a patient who presented two episodes, 9 years apart. The first episode was refractory to plasmapheresis and intravenous immunoglobulin (IVIG) started 2 months after AIBD onset, leading to graft loss. The second episode responded to plasmapheresis, IVIG and rituximab initiated less than 2 weeks after the beginning of symptoms, allowing for long-term recovery. This case suggests that intensive treatment with minimum delay after symptoms onset could sponsor a better evolution.

Keywords: Antibody-induced bile salt export pump deficiency; BSEP; Liver transplantation; PFIC 2; Recurrence.

Publication types

  • Case Reports

MeSH terms

  • ATP Binding Cassette Transporter, Subfamily B, Member 11
  • Cholestasis, Intrahepatic* / diagnosis
  • Cholestasis, Intrahepatic* / etiology
  • Cholestasis, Intrahepatic* / therapy
  • Humans
  • Immunoglobulins, Intravenous
  • Liver Transplantation* / adverse effects
  • Plasmapheresis
  • Rituximab / therapeutic use

Substances

  • Rituximab
  • ATP Binding Cassette Transporter, Subfamily B, Member 11
  • Immunoglobulins, Intravenous