Diagnosis and Management of Primary Sclerosing Cholangitis

Abstract

Primary sclerosing cholangitis (PSC) is a rare, idiopathic, and complex disease entity that affects both intrahepatic and extrahepatic biliary ducts. The exact cause of this disease is not known; however, it may be due to an autoimmune process due to its link to ulcerative colitis. PSC is a disease that may present with varying symptomatology over an extended period of time. Workup includes standard liver function blood tests as well as a right upper quadrant ultrasound to evaluate the biliary ducts. Abnormalities of the biliary tree on ultrasound prompts further investigation with a magnetic resonance cholangiopancreatography (MRCP) and eventual endoscopic retrograde cholangiopancreatography (ERCP). Visualized diffuse biliary strictures and dilations along with intraductal biopsies confirm the diagnosis of PSC and warrant intervention. Dominant strictures are managed with endoscopic dilations and/or stenting. Patients with mild disease and no dominant strictures can be managed with observation and surveillance for progression. The only known cure for PSC is orthotopic liver transplantation.