Recurrent pyogenic cholangitis

Contributors: Cafer Zorkun M.D., PhD.

Overview
Recurrent pyogenic cholangitis (a.k.a. cholangiohepatitis) is a condition that most commonly affects humans of East Asian origins. It is characterized by recurrent bouts of cholangitis in association with intrahepatic pigment stones and intrahepatic biliary obstruction.

Pathogenesis
In recurrent pyogenic cholangitis the gallstones found within the biliary system are calcium bilirubinate stones or pigmented calcium stones. Calcium bilirubinate stones are prevalent in Asia and very rare in Europe and the United States.

In addition to the presence of these friable concretions of various shapes and sizes within the biliary tree, the bile is often muddy in consistency and contains numerous fine particles of calcium bilirubinate. This differs greatly from cholesterol stones, which are common in Europe and the United States. Pure cholesterol stones contain >96% cholesterol whereas mixed cholesterol stones contain 71.3% cholesterol. The formation of calcium bilirubinate stones in RPC has been attributed to the high incidence of infection with Escherichia coli in the bile. In man, the majority of bilirubin is excreted in the bile as bilirubin glucuronide.

Symptoms
Recurrent pyogenic cholangitis is characterized by recurrent attacks of abdominal pain, fever, and jaundice caused by intrahepatic ductal strictures and calculi.

Diagnostic Findings

 * Centrally dilated bile ducts with peripheral tapering.
 * Biliary duct stones.

Patient #1: MR images from a patient with recurrent pyogenic cholangitis
Images courtesy of RadsWiki

Patient #2
Images courtesy of RadsWiki