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Recurrent pyogenic cholangitis

Hannah Lee, MD
Section Editor
Sanjiv Chopra, MD, MACP
Deputy Editor
Anne C Travis, MD, MSc, FACG, AGAF


Recurrent pyogenic cholangitis is a disease characterized by intrabiliary pigment stone formation, resulting in stricturing of the biliary tree and biliary obstruction with recurrent bouts of cholangitis. Various other names have been used since the first description in 1930, including Hong Kong disease, biliary obstruction syndrome of the Chinese, Oriental cholangitis, and Oriental infestational cholangitis [1]. The lack of a uniform nomenclature complicates direct comparison of studies.


Recurrent pyogenic cholangitis is found almost exclusively in people who live or who have lived in Southeast Asia [2-7]. However, the incidence in the West appears to be increasing due to the immigration of people from endemic countries. It occurs in equal frequency in men and women with a peak prevalence in the third and fourth decades of life, although it can also be seen in patients over the age of 60 [8-11]. It is more common in the rural population and in lower socioeconomic groups.


Recurrent pyogenic cholangitis is characterized by recurrent cholangitis caused by bile stasis and stone formation proximal to biliary strictures. The pathogenesis of the stone formation and biliary abnormalities is incompletely understood. Stone formation occurs de novo within the intrahepatic bile ducts in contrast with the more common pattern of stone formation within the gallbladder seen in patients with more common forms of gallstone-related disease.

Transient portal bacteremia is thought to introduce bacteria into the biliary ducts, initiating a cycle of infection and secondary stone formation which lead to further obstruction and infection. Common organisms cultured from bile include E. coli, Klebsiella, Pseudomonas, and Proteus species and, less frequently, anaerobes, although the culture of multiple organisms is common [8,12]. Abnormal phospholipid metabolism has also been implicated [13]. (See "Inherited disorders associated with conjugated hyperbilirubinemia".)

The bile ducts are markedly abnormal, characterized by extrahepatic and intrahepatic ductal dilatation with focal areas of stricturing in the intrahepatic biliary tree. The biliary wall is fibrotic with inflammatory cell infiltration. The bile is purulent and filled with debris composed of bile pigment, desquamated epithelial cells, bacteria, and pus.


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Literature review current through: Sep 2016. | This topic last updated: Jan 20, 2015.
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