A 62-year-old man presents to his primary care physician complaining of constant itchings and yellow skin and eyes. On exam, there is no evidence of hepatomegaly, however, laboratory findings are of concern for elevated serum ALP and GGT.
Introduction
Chronic cholestatic syndrome characterized by fibrosing inflammation in the intrahepatic and extrahepatic bile duct
onset usually insidious
leads to narrowing and, eventually, obliteration of the bile ducts
Cause of primary sclerosing cholangitis (PSC) is unknown
PSC occurs most often in
young men (< 50 years old)
HLA-DR52a positive
commonly associated with inflammatory bowel disease
especially ulcerative colitis
Presentation
Symptoms
pruritus
jaundice
some present with hepatosplenomegaly or features of cirrhosis
Evaluation
Labs
↑ serum ALP, GGT
bilirubin ↑ with conjugated fraction > 50%
typical of obstructive pattern
no urine urobilinogen
low urine urobilin
Endoscopic retrograde cholangiography (ERCP)
give the biliary tree an irregular beaded appearance
Treatment
Liver transplantation
prior to liver failure is a possibility in selected cases
Prognosis, Prevention, and Complications
Prognosis is poor
May result in liver failure, cirrhosis, cholangiocarcinoma
