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Key Features

Essentials of Diagnosis

  • Progressive jaundice, itching, and other features of cholestasis

  • Males, aged 20–50 years old

  • Often associated with ulcerative colitis

  • Diagnosis based on characteristic cholangiographic findings

  • At least 10% risk of cholangiocarcinoma

General Considerations

  • Characterized by diffuse inflammation of the biliary tract leading to fibrosis and strictures of the biliary system

  • Associated with the histocompatibility antigens HLA-B8 and -DR3 or -DR4

  • Occasional patients have clinical and histologic features of both sclerosing cholangitis and autoimmune hepatitis

  • Cholangitis in IgG4-related disease

    • May be difficult to distinguish from primary sclerosing cholangitis and even cholangiocarcinoma

    • Is associated with autoimmune pancreatitis (see Chronic Pancreatitis)

    • Is responsive to corticosteroids

  • The diagnosis is difficult to make after biliary surgery or intrahepatic artery chemotherapy, which may result in bile duct injury

  • Primary sclerosing cholangitis must be distinguished from idiopathic adulthood ductopenia, a rare disorder affecting young to middle-aged adults who manifest cholestasis resulting from loss of interlobular and septal bile ducts yet who have a normal cholangiogram

Demographics

  • Between 60% and 70% of affected persons are male, usually 20–50 years old (median age 41)

  • Incidence

    • Nearly 3.3 per 100,000 in Asian Americans

    • 2.8 per 100,000 in Hispanic Americans

    • 2.1 per 100,000 in African Americans

    • Intermediate incidence in whites (and increasing)

  • Prevalence: 21 per 100,000 men and 6 per 100,000 women in the United States

  • Closely associated with inflammatory bowel disease (more commonly ulcerative colitis than Crohn colitis), which is present in approximately two-thirds of patients

  • However, clinically significant sclerosing cholangitis develops in only 1–4% of patients with ulcerative colitis

  • As in ulcerative colitis, smoking is associated with a decreased risk of primary sclerosing cholangitis; so is coffee consumption

Clinical Findings

Symptoms and Signs

  • Progressive obstructive jaundice, frequently associated with fatigue, pruritus, anorexia, and indigestion

  • Esophageal varices on initial endoscopy are most likely found in patients with a higher Mayo risk score based on age, bilirubin, albumin, and AST and a higher AST/ALT ratio

  • New varices are likely to develop in those with a lower platelet count and higher bilirubin at 2 years

  • In patients with primary sclerosing cholangitis, ulcerative colitis is frequently characterized by rectal sparing and backwash ileitis

  • Associations with cardiovascular disease and diabetes mellitus have been reported

  • Women with primary sclerosing cholangitis may be more likely to have recurrent urinary tract infections

Differential Diagnosis

  • Primary biliary cholangitis

  • Choledocholithiasis

  • Cancer of pancreas or biliary tract

  • Biliary stricture

  • Drug-induced cholestasis, eg, chlorpromazine

  • Inflammatory bowel disease complicated by cholestatic liver disease

  • Idiopathic adulthood ductopenia

  • Clonorchis sinensis (Chinese liver fluke)

  • Fasciola hepatica (sheep liver fluke)

  • Sclerosing cholangitis due to

    • Cytomegalovirus

    • Cryptosporidiosis

    • Microsporidiosis (in AIDS)

Diagnosis

Laboratory Tests

  • Condition may be diagnosed in the presymptomatic phase because of an elevated alkaline phosphatase ...

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