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Rituximab as therapy for the recurrence of bile salt export pump deficiency after liver transplantation
Author(s) -
Lin Henry C.,
Alvarez Luis,
Laroche Greggy,
MelinAldana Hector,
Pfeifer Kim,
Schwarz Kathleen,
Whitington Peter F.,
Alonso Estella M.,
Ekong Udeme D.
Publication year - 2013
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.23754
Subject(s) - bile salt export pump , medicine , progressive familial intrahepatic cholestasis , liver transplantation , rituximab , cholestasis , plasmapheresis , liver disease , antibody , gastroenterology , transplantation , immunology , biochemistry , chemistry , transporter , gene
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. Liver Transpl 19:1403‐1410, 2013 . © 2013 AASLD.