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

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...

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Detalles Bibliográficos
Autores: Quintero, Jesús|||0000-0002-6456-9031, Juamperez, Javier|||0000-0002-8556-6374, Gonzales, Emmanuel|||0000-0002-1546-3193, Julio, Ecaterina|||0000-0002-5678-7370, Mercadal-Hally, Maria|||0000-0003-1687-6448, Collado-Hilly, Mauricette|||0000-0002-3225-086X, Marín-Sánchez, Ana|||0000-0003-2873-456X, Charco, Ramon|||0000-0001-9709-6288
Tipo de recurso: artículo
Fecha de publicación:2020
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:227683
Acceso en línea:https://ddd.uab.cat/record/227683
https://dx.doi.org/urn:doi:10.5223/pghn.2020.23.2.174
Access Level:acceso abierto
Palabra clave:Cholestasis, progressive familial intrahepatic 2
Liver transplantation
Child
Descripción
Sumario: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.

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