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

Henry 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 journalArticle

19 Citations (Scopus)

Abstract

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
Volume19
Issue number12
DOIs
StatePublished - Dec 1 2013
Externally publishedYes

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Bile Acids and Salts
Liver Transplantation
Recurrence
Antibodies
End Stage Liver Disease
Plasmapheresis
Intravenous Immunoglobulins
Cholestasis
Therapeutics
Immunosuppressive Agents
Allografts
Anti-Idiotypic Antibodies
Immunoglobulin G
Adenosine Triphosphate
Transplantation
Monoclonal Antibodies
Transplants
Mutation
Rituximab
Progressive familial intrahepatic 2 Cholestasis

ASJC Scopus subject areas

  • Surgery
  • Hepatology
  • Transplantation

Cite this

Lin, H., Alvarez, L., Laroche, G., Melin-Aldana, H., Pfeifer, K., Schwarz, K., ... Ekong, U. D. (2013). Rituximab as therapy for the recurrence of bile salt export pump deficiency after liver transplantation. Liver Transplantation, 19(12), 1403-1410. https://doi.org/10.1002/lt.23754

Rituximab as therapy for the recurrence of bile salt export pump deficiency after liver transplantation. / Lin, Henry; Alvarez, Luis; Laroche, Greggy; Melin-Aldana, Hector; Pfeifer, Kim; Schwarz, Kathleen; Whitington, Peter F.; Alonso, Estella M.; Ekong, Udeme D.

In: Liver Transplantation, Vol. 19, No. 12, 01.12.2013, p. 1403-1410.

Research output: Contribution to journalArticle

Lin, H, Alvarez, L, Laroche, G, Melin-Aldana, H, Pfeifer, K, Schwarz, K, Whitington, PF, Alonso, EM & Ekong, UD 2013, 'Rituximab as therapy for the recurrence of bile salt export pump deficiency after liver transplantation', Liver Transplantation, vol. 19, no. 12, pp. 1403-1410. https://doi.org/10.1002/lt.23754
Lin, Henry ; Alvarez, Luis ; Laroche, Greggy ; Melin-Aldana, Hector ; Pfeifer, Kim ; Schwarz, Kathleen ; Whitington, Peter F. ; Alonso, Estella M. ; Ekong, Udeme D. / Rituximab as therapy for the recurrence of bile salt export pump deficiency after liver transplantation. In: Liver Transplantation. 2013 ; Vol. 19, No. 12. pp. 1403-1410.
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