Recurrent Pyogenic Cholangitis 9/3/2018

Paula presented a case of an elderly Mexican woman presenting with 2-4 weeks of non-specific complaints i.e. abdominal pain, nausea, anorexia, and yellow discoloration. Her labs were notable for obstructive pattern LFT abnormalities. US revealed cirrhosis, and CT AP revealed dilated stone-filled intrahepatic and extrahepatic biliary ducts. ERCP later revealed innumerable stones and copious sludge throughout the intrahepatic ducts, common hepatic ducts, and CBD. Her presentation was consistent with recurrent pyogenic cholangitis, also known as…

  • Hong Kong Disease
  • Oriental cholangitis
  • Oriental cholangiohepatitis
  • Oriental infestational cholangitis
  • Biliary obstruction syndrome of the Chinese
  • Cholangiohepatitis

I’m not kidding.

Epidemiology:

  • Found primarily in residents of East and SE Asia, or people who have resided there.
  • Associated with poorer socioeconomic backgrounds

Pathophysiology/Presentation

  • Characterized by recurrent cholangitis, biliary stasis, and innumerable intrabiliary stone formation ) calcium bilirubinate stones) throughout a fibrotic biliary ductal system.
    • Stone formation can occur within the intrahepatic bile ducts unlike the most common presentation of stone formation within the gallbladder.
  • Possibly associated with biliary parasitosis, namely liver flukes like Clonorchis sinensis, Fasciola spp. Ascaris lumbricoides (from prior studies revealing dead parasites within the stones).
  • Leads to recurrent infections due to persistent biliary stasis, stone formation, and bile duct structures.
  • Pts often present with cholangitis, non-spec abd pain, or pancreatitis. Often unrecognized at first, and recurrent attacks leads to progressive biliary and hepatic damage which may lead to abscesses or cirrhosis.

Diagnosis

  • Imaging correlating with clinical history, US first, CT, MRCP (can evaluate extent of biliary involvement), percutaneous transhepatic cholangioscopy
  • Invasive: ERCP
  • Abnormalities commonly found: intra + extrahepatic duct dilatation, periductal fibrosis, missing duct sign (complete obstruction)
  • All pts should have O&P checked

Management

  • Complicated, combination of management of infection and biliary drainage.
  • Severe cases: resection of affected bile duct segments followed by biliary-enteric anastomosis (i.e. hepaticojejunostomy, may require partial hepatectomy)
  • No optimal strategies have been established by large studies.

Big picture

  • Predominantly in Asians
  • Unclear pathophysiology but thought to be somewhat related to parasites
  • Lack of large studies on optimal management.
  • One Taiwanese study: 7% pts develop cirrhosis, 3% with cholangiocarcinoma

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