Recurrent Pyogenic Cholangitis 

  • Author: Willis Parsons, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Feb 23, 2010
 

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.

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Pathophysiology

Recurrent episodes of cholangitis develop as a result of pyogenic bile duct infection. Predominantly coliform-type bacteria are seeded to 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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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.

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.

Mortality/Morbidity

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.

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Contributor Information and Disclosures
Author

Willis Parsons, MD  Medical Director of the GI Center, Northwest Community Hospital

Willis Parsons, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Specialty Editor Board

Anil Minocha, MD, FACP, FACG  Clinical Professor, School of Pharmacy, Professor of Medicine, Director of Digestive Diseases, Medical Director of Nutrition Support, Medical Director of Gastrointestinal Endoscopy, Internal Medicine Department, University of Mississippi Medical Center

Anil Minocha, MD, FACP, FACG is a member of the following medical societies: American Academy of Clinical Toxicology, American Association for the Study of Liver Diseases, American College of Forensic Examiners, American College of Gastroenterology, American College of Physicians, American Federation for Clinical Research, American Gastroenterological Association, and American Society of Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

James L Achord, MD  Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine

James L Achord, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, American Society for Gastrointestinal Endoscopy, Mississippi State Medical Association, New York Academy of Sciences, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

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