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

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 presentati…