Recurrent pyogenic cholangitis

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Recurrent pyogenic cholangitis (a.k.a. cholangiohepatitis, Hong Kong disease, oriental chlangitis, oriental cholangiohepatitis, biliary obstruction syndrome of the Chinese, oriental cholangitis)[1] is characterised by recurrent bouts of bacterial cholangitis with primary hepatolithiasis.[1] It is prevalent in Hong Kong and East Asian including China, Taiwan, Korea, Japan, Indonesia and the Philippines.[1] Apart from affecting humans it is also a common disease in cats.

Clinical manifestations[edit]

Presentation can be atypical with no pain or fever especially in the elderly population.[1] Hepatolithiasis may present with biliary colic, acute pancreatitis, obstructive jaundice and less commonly, hepatomegaly and abnormal liver chemistry.[1] Chronic biliary obstruction may cause jaundice, pruritus, liver abscess, and liver atrophy, mostly affecting the left lobe and the left lateral segment of the liver, and eventually secondary biliary cirrhosis and cholangiocarcinoma.[2][3][4][5]


Suppurative cholangitis, liver abscess, empyema of the gallbladder, acute pancreatitis, thrombophlebitis of hepatic or portal veins, and septicemia are acute complications of the disease, to which patients may succumb during the acute attacks.[1]

Chronically, complications include cholangiocarcinoma and intraductal papillary neoplasm.[1]


In RPC 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 humans, the majority of bilirubin is excreted in the bile as bilirubin glucuronide.

Hepatolithiasis is associated with Clonorchis sinensis and Ascaris lumbricoides infestation of the liver. This theory is based on high incidence of dead parasites or ova within stone in autopsy findings.[6][7]

See also[edit]


  1. ^ a b c d e f g Tsui, Wilson; Chan, Yiu-kay; Wong, Chi-tat; Lo, Yan-fai; Yeung, Yat-wah; Lee, Yat-wing. "Hepatolithiasis and the Syndrome of Recurrent Pyogenic Cholangitis: Clinical, Radiologic, and Pathologic Features". Seminars in Liver Disease. 31 (01): 033–048. doi:10.1055/s-0031-1272833. PMID 21344349. 
  2. ^ Caroli, J. (1973-01-01). "Diseases of the intrahepatic biliary tree". Clinics in Gastroenterology. 2 (1): 147–161. ISSN 0300-5089. PMID 4594323. 
  3. ^ Lim, J. H. (1991-07-01). "Oriental cholangiohepatitis: pathologic, clinical, and radiologic features". AJR. American journal of roentgenology. 157 (1): 1–8. doi:10.2214/ajr.157.1.2048504. ISSN 0361-803X. PMID 2048504. 
  4. ^ Fan, S. T.; Choi, T. K.; Lo, C. M.; Mok, F. P.; Lai, E. C.; Wong, J. (1991-04-01). "Treatment of hepatolithiasis: improvement of result by a systematic approach". Surgery. 109 (4): 474–480. ISSN 0039-6060. PMID 2008653. 
  5. ^ Lee, W. J.; Lim, H. K.; Jang, K. M.; Kim, S. H.; Lee, S. J.; Lim, J. H.; Choo, I. W. (2001-10-01). "Radiologic spectrum of cholangiocarcinoma: emphasis on unusual manifestations and differential diagnoses". Radiographics: A Review Publication of the Radiological Society of North America, Inc. 21 Spec No: S97–S116. doi:10.1148/radiographics.21.suppl_1.g01oc12s97. ISSN 0271-5333. PMID 11598251. 
  6. ^ Chou, S. T.; Chan, C. W. (1980-07-01). "Recurrent pyogenic cholangitis: a necropsy study". Pathology. 12 (3): 415–428. doi:10.3109/00313028009077105. ISSN 0031-3025. PMID 7432820. 
  7. ^ Caroli, J. (1973-01-01). "Diseases of the intrahepatic biliary tree". Clinics in Gastroenterology. 2 (1): 147–161. ISSN 0300-5089. PMID 4594323.