A 27-year-old man presents to a dermatologist because his skin has become chronically itchy. He has also been experiencing chronic, progressive fatigue. On physical examination, no specific skin lesions are seen, but the patient is noted to be mildly jaundiced. Serum chemistry tests demonstrate elevated serum alkaline phosphatase and bilirubin with minimally increased transaminases. He was fuher referred to a surgeon. Endoscopic retrograde cholangiography demonstrates multiple sho strictures and saccular dilatations involving the intrahepatic and extrahepatic bile ducts. Which of the following is the most likely diagnosis?
This presentation is typical for primary sclerosing cholangitis, which is an inflammatory disease that affects the bile duct system, most often in young men. Most of the findings are fairly nonspecific, but the characteristic cholangiographic picture illustrated is the clue that gives the diagnosis, and will probably be mentioned in any case history about the disease that you encounter. Ascending cholangitis is a bacterial infection of the bile duct system, and does not produce the characteristic sacculations of primary sclerosing cholangitis. A bile duct tumor can produce a diffuse dilation of the biliary tree above it, secondary to back-pressure effects, but would not produce the alternating strictures and sacculations seen in this case. Primary biliary sclerosis affects the intrahepatic, but not the extrahepatic bile ducts.
Ascending cholangitis
Bile duct tumor
Primary biliary sclerosis
Primary sclerosing cholangitis