Although at presentation, most patients with this disease are asymptomatic, this ERCP is from a patient with inflammatory bowel disease and an elevated alkaline phosphatase. She initially presented with fatigue and pruritis, though other symptoms include fever, chills, night sweats, and RUQ pain. Alkaline phosphatase is remarkably elevated, though AST/ALT are less than 300. She also has hypoalbuminemia, hypergammaglobulinemia, increased serum IgM. Antimicrosomal antibody is negative though a smattering of other autoimmune markers are positive.Challenge: What's your most likely diagnosis?
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Primary Sclerosing Cholangitis
Primary biliary cirrhosis
primary sclerosing cholangitis..
ah yes, i always mix up PSC and PBC, but this one happens to be PSC (negative antimicrosomal antibody, association with IBD)
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ERCP
The ERCP shows intra- and extrahepatic biliary strictures (thin arrow) and dilations (thick arrow) which, along with the clinical scenario, suggests primary sclerosing cholangitis. This is a disease of unknown etiology characterized by inflammation, fibrosis, and stricturing of medium and large sized ducts in the intra- and extrahepatic biliary tree. There is a strong association with ulcerative colitis. Eventually, it can lead to end-stage liver disease and portal hypertension.
Sources: UpToDate; nature.com
I think you meant in the question that anti-mitochondrial antibody is negative not anti-microsomal antibody
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