Recurrent pyogenic cholangitis
Recurrent pyogenic cholangitis (RPC), also known as Hong Kong disease, Oriental cholangitis, and Oriental infestational cholangitis, is a chronic infection characterized by recurrent bouts of bacterial cholangitis with primary hepatolithiasis. It is exclusive to people who live or have lived in southeast Asia. Presentation Presentation can be atypical and without pain or fever, especially in the elderly. Positive symptoms include biliary colic, acute pancreatitis, obstructive jaundice and less commonly, liver enlargement and abnormal liver function tests. Additional complications in the acute setting include ascending cholangitis, gallbladder empyema, clotting within the hepatic and portal veins, sepsis and death. Chronic biliary obstruction may cause jaundice, itchiness, liver abscesses, and cirrhosis, particularly at the left lobe segment 3, and can eventually lead to intraductal papillary mucinous neoplasm or cholangiocarcinoma. Pathogenesis With RPC, the gallstones found within the biliary system are made of calcium bilirubinate or pigmented calcium. Calcium bilirubinate stones are prevalent in Asia and very rare in Europe and the United States. They tend to be friable concretions of various shapes and sizes within the biliary tree, and their associated bile is often muddy in consistency and contains numerous fine particles of calcium bilirubinate as well. This differs greatly from cholesterol stones,