Sclerosing Cholangitis

INTRODUCTION Sclerosing cholangitis represents an array of chronic, cholestatic diseases of the intrahepatic and extrahepatic bile duct systems. Clinically, this syndrome is characterized by progressive fatigue, pruritus, and in later stages, by abdominal pain, recurrent fever, and jaundice. These symptoms are resultant from inflammatory and fibrosing obstruction of the biliary system. The progressive medical course of sclerosing cholangitis can lead to destruction of the bile ducts and advancement to biliary cirrhosis, portal hypertension, cholangiocarcinoma, and liver failure. Premature death is frequently inevitable for patients without liver transplantation. Sclerosing cholangitis can be due to primary or secondary disorders of the biliary tree. Primary sclerosing cholangitis (PSC) is idiopathic. PSC is typically a chronic, progressive disorder which is often refractory to medical therapy. This condition frequently occurs in association with inflammatory bowel disease (IBD), with only about 20% of patients not having any evidence of IBD.1 The majority of patients with PSC are found to have a concomitant diagnosis of ulcerative colitis (UC). Conversely, it has been estimated that PSC occurs in approximately 5%-10% of patients with UC, whereas the prevalence is lower in patients with Crohn’s disease.2 Secondary sclerosing cholangitis (SSC) is clinically comparable to PSC, yet is caused by known disorders that afflict insult to the biliary tree. Examples of these processes include choledocholithiasis, bile duct malignancy, congenital bile duct abnormalities such as Caroli’s disease, biliary ischemia from hepatic arterial occlusion, chemotherapeutic agents (i.e., 5-fluorouracil) and acquired immunodeficiency syndrome (AIDS)-related cholangiopathy (Table 1).1