MRCP pattern: Multifocal strictures with intervening dilatation that beads the whole biliary tree equal primary sclerosing cholangitis.
In PSC, repeated inflammation and fibrosis pinch the bile ducts at many points, both inside and outside the liver. Between the pinches the ducts dilate, so the cholangiogram looks like a string of beads. The typical patient is a young or middle-aged man, often with ulcerative colitis, who has recurrent right-upper-quadrant pain, intermittent jaundice, and fatigue. Cholangiography (MRCP first, ERCP if needed) is the cornerstone of diagnosis.
Eliminate the rest: Primary biliary cholangitis attacks tiny ducts in middle-aged women and is AMA-positive, with no large-duct beading. Caroli's disease shows ballooned cystic intrahepatic ducts with the central dot sign. Oriental cholangitis shows ducts crammed with pigment stones and is endemic to East Asia. The beaded large-duct pattern fits only PSC.
Ref: Bailey and Love, 27th edition.