Recurrent Pyogenic Cholangitis

Updated: Jul 24, 2015
  • Author: Praveen K Roy, MD, AGAF; Chief Editor: Julian Katz, MD  more...
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Overview

Background

Cholangiohepatitis, or recurrent pyogenic cholangitis (RPC), is characterized by a recurrent syndrome of bacterial cholangitis that occurs in association with intrahepatic pigment stones and intrahepatic biliary obstruction. It was first described in 1930 and has been called by different names, including Hong Kong disease, Oriental cholangitis, and Oriental infestational cholangitis.

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Pathophysiology

Recurrent episodes of cholangitis develop as a result of pyogenic bile duct infection. Predominantly coliform-type bacteria are seeded into the biliary system, and, as a consequence, a cascade of stone formation and recurrent cholangitis is initiated. The bile ducts of patients with cholangiohepatitis are thought to be increasingly susceptible to bacterial seeding as a result of helminthic infections or as a consequence of malnutrition.

Malnutrition results in a relative deficiency of enzymes, such as beta-glucuronidase inhibitor glucaro-1,4-lactone, that inhibit deconjugation of conjugated bilirubin. The relatively unopposed deconjugation favors breakdown of conjugated bilirubin to its unconjugated form, which then complexes with calcium to form calcium bilirubinate. Calcium bilirubinate acts as the nidus for the formation of pigmented stones. Pigment stone formation then leads to recurrent biliary obstruction with the characteristic consequence of suppurative cholangitis. This theory of nutrition associated deficiency of beta-glucuronide has been called into question as definite differences in enzyme levels between patients with gallstones and control subjects are difficult to demonstrate.

Infection in the biliary system from Ascaris lumbricoides or from trematodes, such as Clonorchis sinensis and Opisthorchis viverrini, often results in significant epithelial damage. Clinical and experimental data suggest that coliforms may then result in portal bacteremia by bacterial translocation as a result of this epithelial damage. Repeated portal bacteremia, in turn, results in the cascade of events characterized by biliary stasis, obstruction, and stone formation, which consequently result as RPC.

However, it is more likely that infection and malnutrition act in concert to give rise to RPC.

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Etiology

The underlying mechanism of RPC is unclear. Most experts believe that RPC is initiated by helminthic infection of the bile ducts and/or sludge/stone formation from deficient glucuronidation as a consequence of profound malnutrition. The initial insult(s) to the bile ducts precipitates a cycle of biliary stone formation and infection that results in recurrent episodes of pyogenic cholangitis.

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Epidemiology

Frequency

United States

The condition is rare. It often is observed in immigrants from Southeast Asia or from other regions of the world where trematode infections are endemic. Patients who have RPC develop it over time; hence, there does not appear to be a risk of developing RPC for visitors to endemic areas.

International

The worldwide prevalence of cholangiohepatitis is unclear largely because of the paucity of disease statistics in many parts of the world where this disease is endemic. RPC is prevalent in Hong Kong and East Asia. A case in Turkey was successfully treated with surgery. [1]

Although the arrival of Asian immigrants in the United Kingdom, Australia, Europe, and the United States has resulted in more cases reported in nonendemic areas, the overall incidence in East Asia has been in decline over the last 20 years. Population surveys confirm this trend. The decline in incidence has been credited to the improved economic situation and living standards with the associated westernization of diet.

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Prognosis

The prognosis is variable and is directly related to the presence or absence of comorbidities, the presence or absence of liver dysfunction, and the presence or absence of malignancy. In general, the prognosis of patients with cholangiohepatitis is not well documented; however, death occurs in approximately 15-20% of patients over 5-6 years.

Morbidity/mortality

Morbidity is related to recurrent biliary infection and may manifest as pancreatobiliary abnormalities, such as pancreatitis, biliary fistulae, hepatic abscesses, and suppurative cholangitis. Consequences of systemic infections and remote foci of infections account for distant or nonpancreatobiliary morbidity.

Complications

The complications of RPC are protean; acutely, patients may experience systemic sepsis with all of the potential complications of single and multisystem organ failure and hemostatic dysfunction. Over time, patients may develop cirrhosis with portal hypertension and parenchymal insufficiency. Cholangiocarcinoma is an increasingly well-recognized long-term complication of cholangiohepatitis.

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