primary sclerosing cholangitis

• idiopathic
  • a/w IBD (50%), usually UC
• M > F
• peak age 20-30 y/o

• chronic inflammation + fibrosis of all bile ducts
• stricture, obstruction, abscess ("string of beads")
• intramural pseudo-diverticula

• cholangioCa (10%!!)
  • dilatation proximal to stricture may signal Ca

DDx:

• cholangioCa (diffuse form)
• primary biliary cirrhosis (PBC)
• AIDS-related cholangitis (? d/t CMV)

© 2002, C.E. Kahn, Jr.
2002-04-18