Review Question - QID 106582

This patient is presenting with a cholestatic pattern, a medical history of inflammatory bowel disease (IBD), and an ERCP that demonstrates intermittent strictures. These findings are highly suggestive of Primary Sclerosing Cholangitis (PSC), which on pathology will demonstrate obliteration of both intra- and extra-hepatic bile ducts.

PSC is a chronic cholestatic liver disease characterized by inflammation, destruction, fibrosis, progressive narrowing, and saccular dilatations of the bile ducts. PSC is an autoimmune condition that is closely linked with IBD, in particular ulcerative colitis. Presentation will demonstrate a cholestatic pattern (elevated GGT and Alkaline Phosphatase); however, hyperbilirubinemia is rare. Elevation in AST and ALT are also common primary findings. Definitive diagnosis is obtained through imaging of the biliary tree.

Eaton et al. discuss the pathogenesis of PSC. PSC involves chronic inflammation and cholestasis of the biliary epithelium, resulting in multifocal bile duct strictures that can affect the entire biliary tree. Over time, hepatic involvement can result in cirrhosis or malignancy. To date, there are few effective therapeutic options, and cirrhosis can lead to the need for liver transplantation.

Chandok et al. discuss the management of PSC. As previously stated, there are few available treatment modalities, other than liver transplantation. The authors' recommend the use of therapeutic cholangiography with dilation or stent insertion for episodes of cholangitis with dominant strictures. Additionally, the literature is mixed on the use of Ursodeoxycholic Acid, and the authors recommend its use with thoughtful consideration.

Figure A is an ERCP demonstrating the characteristic strictures and sacculations which are pathognomonic for PSC. This is the typical "string of beads" finding. Illustration A is a liver biopsy specimen with H&E stain showing a portal triad that contains a degenerating bile duct (double arrow) that is surrounded by lymphoid follicles and a granuloma containing a giant cell (single arrow), as commonly found in primary biliary cirrhosis (PBC).

Incorrect Answers:
Answer 1: Porcelain gallbladder is a rare manifestation of chronic cholecystitis, characterized by intramural calcification of the gallbladder wall. It would not commonly cause a cholestatic pattern on serum analysis.
Answers 2 and 5: Elevated serum anti-mitochondrial antibodies and granulomatous inflammation of intrahepatic bile ducts on biopsy (Illustration A) are highly suggestive of primary biliary cirrhosis (PBC). Of note, PBC has no association with inflammatory bowel disease.
Answer 4: Autoimmune hepatitis can present with transaminitis and right upper quadrant tenderness. Elevations in anti-smooth muscle antibodies can be found.