Primary Sclerosing Cholangitis

A 45-year-old man presented with a 2-month history of progressive fatigue, weight loss of 10 lb, abdominal pain, and pruritus. The patient had been taking a maintenance dose of oral mesalamine since he received a diagnosis of ulcerative colitis 14 years before.

The physical examination was remarkable for jaundice and right upper quadrant abdominal pain. Laboratory evaluation revealed mild leukocytosis, anemia (hemoglobin level, 11 g/dL), cholestatic liver function tests (alkaline phosphatase, 546 U/mL; bilirubin, 3.1 mg/dL), and mildly elevated levels of aminotransferases. The endoscopic retrograde cholangiopancreatography (ERCP), pictured at right, disclosed multifocal, diffusely distributed strictures of the intrahepatic and extrahepatic biliary tree-a finding consistent with sclerosing cholangitis.

Dr Klaus E. Mnkmller of Birmingham, Ala, writes that primary sclerosing cholangitis is a chronic cholestatic liver disease that often progresses to cirrhosis. The characteristic pathologic feature of this condition is inflammation, with obliterative fibrosis of the intrahepatic and extrahepatic bile ducts. Sclerosing cholangitis frequently occurs in patients with ulcerative colitis and, less commonly, in those with Crohn's disease.

Most patients who have this disease are male, with a mean age at diagnosis of 45 years. The clinical presentation can vary from asymptomatic disease to end-stage cirrhosis. Most patients complain of pruritus and fatigue; up to 25% have fever and cholangitis. Common laboratory test findings include a markedly elevated alkaline phosphatase level, direct hyperbilirubinemia, and mild elevation of the aminotransferases.

ERCP is the most important diagnostic study for sclerosing cholangitis; typical findings include multifocal stricturing and irregularity of the intrahepatic and extrahepatic biliary tree. These strictures usually are diffuse, short, and annular, with intervening segments of normal to dilated ducts giving a beaded (“rosary bead”) appearance.

Treating the complications of the disease is the mainstay of therapy. This patient's pruritus improved significantly with oral cholestyramine. He has remained symptom-free at 10 months' follow-up. Cholangitis is managed with antibiotics, and a dominant biliary stricture may be palliated with the placement of plastic stents. Liver test results and symptoms may improve with ursodeoxycholic acid therapy.¹ Liver transplantation is reserved for patients with end-stage primary sclerosing cholangitis.

REFERENCE:1. Lindor KD, Dickson ER, Baldus WP, et al. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis. Gastroenterology. 1994;106:1284-1290.