Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
Quintero, Jesús (Hospital Universitari Vall d'Hebron)
Juamperez, Javier (Hospital Universitari Vall d'Hebron)
Gonzales, Emmanuel (Inserm Unité Mixte de Recherche 1193, Université Paris-Saclay)
Julio, Ecaterina (Hospital Universitari Vall d'Hebron)
Mercadal-Hally, Maria (Hospital Universitari Vall d'Hebron)
Collado-Hilly, Mauricette (Inserm Unité Mixte de Recherche 1193, Université Paris-Saclay)
Marín-Sánchez, Ana (Hospital Universitari Vall d'Hebron)
Charco, Ramon (Hospital Universitari Vall d'Hebron)
Universitat Autònoma de Barcelona

Date:	2020
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.
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Subject:	Cholestasis, progressive familial intrahepatic 2 ; Liver transplantation ; Child
Published in:	Pediatric Gastroenterology, Hepatology & Nutrition, Vol. 23 (march 2020) , p. 174-179, ISSN 2234-8840

DOI: 10.5223/pghn.2020.23.2.174
PMID: 32206630

6 p, 1.1 MB

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