Rituximab as therapy for the recurrence of bile salt export pump deficiency after liver transplantation

Henry C. Lin, Luis Alvarez, Greggy Laroche, Hector Melin-Aldana, Kim Pfeifer, Kathleen Schwarz, Peter F. Whitington, Estella M. Alonso, Udeme D. Ekong

Research output: Contribution to journalArticlepeer-review

22 Scopus citations


Progressive familial intrahepatic cholestasis type 2 (PFIC2) results from recessive mutations in the adenosine triphosphate-binding cassette B11 gene, which encodes for bile salt export pump (BSEP). Liver transplantation (LT) is offered to PFIC2 patients with end-stage liver disease. Reports have described recurrent cholestasis in PFIC2 patients after transplantation, and this has been associated with immunoglobulin G antibodies to BSEP. High-titer anti-BSEP antibodies appear to correlate with episodes of cholestatic graft dysfunction. There is no established paradigm for treating antibody-mediated posttransplant BSEP disease. It appears to be refractory to changes in immunosuppressant medications that would typically be effective in treating allograft rejection. Taking what is known about its pathophysiology, we designed a treatment consisting of rituximab, a chimeric monoclonal anti-CD20 antibody, in combination with intravenous immunoglobulin and plasmapheresis. Using this approach, we report the successful management of 2 patients with antibody-mediated recurrence of PFIC2 after LT.

Original languageEnglish (US)
Pages (from-to)1403-1410
Number of pages8
JournalLiver Transplantation
Issue number12
StatePublished - Dec 2013

ASJC Scopus subject areas

  • Surgery
  • Hepatology
  • Transplantation


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