Z Gastroenterol 2016; 54 - KV275
DOI: 10.1055/s-0036-1587051

Plasma separation and anion adsorption results in rapid improvement of nasobiliary drainage (NBD)-refractory pruritus in BRIC type 2

R Holz 1, G Christidis 1, JK Walther 1, M Reichert 1, R Liebe 1, S Seiler-Mussler 2, S Zewinger 2, U Sester 2, M Schuster 1, RM Bohle 3, HE Wasmuth 4, F Lammert 1, M Krawczyk 1
  • 1Klinik für Innere Medizin II, Universitätsklinikum des Saarlandes, Homburg, Deutschland
  • 2Klinik für Innere Medizin IV, Universitätsklinikum des Saarlandes, Homburg, Deutschland
  • 3Institut für Allgemeine und Spezielle Pathologie, Universitätsklinikum des Saarlandes, Homburg, Deutschland
  • 4Medizinische Klinik, Luisenhospital, Aachen, Deutschland

Introduction: Benign recurrent intrahepatic cholestasis (BRIC) is a rare genetic disease caused by mutations in the hepatobiliary transporters for aminophospholipids (ATP8B1, type 1) or bile salts (ABCB11, type 2). It is characterized by episodes of cholestatic itch and jaundice. Nasobiliary drainage (NBD) and extracorporeal blood purification are considered as rescue therapies for refractory pruritus (Hegade VS, Krawczyk M et al. Aliment Pharmacol Ther 2016). Here we present a case of an NBD-refractory BRIC type 2 patient who improved rapidly under plasma separation and anion absorption therapy.

Case presentation: The 23-year-old male patient was referred to our department with a prolonged episode of refractory pruritus due to BRIC type 2. He is a compound heterozygous carrier of the ABCB11 mutations c.3491delT and c.3826C>T. It was his 4th BRIC episode, most likely triggered by a viral infection, and lasted for several weeks. During this period he underwent therapy with ursodeoxycholic acid, rifampicin, and NBD, without improvement of itch. At admission to our department he suffered from pruritus intensity 7/10 on visual analog scale (VAS). Serum bilirubin concentration was 27.6 mg/dl and AP activity was 342 U/l (norm < 129 U/l) but GGT was normal, consistent with BRIC type 2. Given the refractory pruritus, we performed extracorporal blood purification with plasma separation and anion adsorption (Plasorba BR-350, Diamed). This resulted in an improvement of pruritus already on the first day (i.e. VAS 4/10). Overall, 9 procedures were performed. Pruritus decreased to 1/10 and bilirubin was < 10.0 mg/dl. The patient was discharged without pruritus on day 15. Liver biopsy showed bland cholestasis but neither cirrhosis nor vanishing bile duct syndrome.

Discussion: Therapy of pruritus in BRIC patients is troublesome. Hence invasive techniques have been advocated to relieve pruritus in these patients. Since BRIC2 is caused by dysfunction of the hepatobiliary bile salt export pump and cessation of bile salt-dependent bile flow, we reckon that NBD placement fails to improve the cholestasis in this subgroup of BRIC patients. For BRIC type 2, we recommend that extracorporal blood purification, and not NBD, should be the treatment of choice for refractory itch.