Pediatr Gastroenterol Hepatol Nutr.  2020 Mar;23(2):174-179. 10.5223/pghn.2020.23.2.174.

Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient

• Quintero J
• Juamperez
• Gonzales E
• Julio E
• Mercadal-Hally M
• Collado-Hilly M
• Marín-Sánchez A
• Charco R

• KMID: 2471627
• DOI: http://doi.org/10.5223/pghn.2020.23.2.174

Abstract

We present an 8 years old girl who was diagnosed at 6 months of age of Progressive Familial Intrahepatic Cholestasis type 2. Although liver transplantation (LT) was classically considered curative for these patients, cholestasis recurrence with normal gamma-glutamyl transpeptidase (GGT), mediated by anti-bile salt export pump (BSEP) antibodies after LT (auto-antibody Induced BSEP Deficiency, AIBD) has been recently reported. Our patient underwent LT at 14 months. During her evolution, patient presented three episodes of acute rejection. Seven years after the LT, the patient presented pruritus with cholestasis and elevation of liver enzymes with persistent normal GGT. Liver biopsy showed intrahepatic cholestasis and giant-cell transformation with very low BSEP activity. Auto-antibodies against BSEP were detected therefore an AIBD was diagnosed. She was treated with Rituximab and immunoadsorption with resolution of the AIBD. As a complication of the treatment she developed a pneumocystis infection successfully treated with corticoids, cotrimoxazol and anidulafungin.

Keyword

Cholestasis, progressive familial intrahepatic 2; Liver transplantation; Child

MeSH Terms

Adrenal Cortex Hormones
Antibodies
Bile*
Biopsy
Child
Cholestasis
Cholestasis, Intrahepatic
Female
gamma-Glutamyltransferase
Humans
Liver Transplantation
Liver*
Pneumocystis Infections
Pruritus
Recurrence
Rituximab*
Adrenal Cortex Hormones
Antibodies
Rituximab
gamma-Glutamyltransferase

Figure

• 

  Fig. 1 Patient' serum alloantibodies are directed against BSEP. Both anti-GFP antibodies (green) and patient's serum (1/200, red) stained BSEP at the canalicular level of BSEPWT-GFP transfected Can 10 cells. * indicated a canalicular structure. Bar=10 μm.BSEP: bile salt export pump, GFP: green fluorescent protein.

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