eMedicine Specialties > Emergency Medicine > Gastrointestinal

Cholangitis

Author: Adam J Rosh, MD, MS, Assistant Professor, Department of Emergency Medicine, Wayne State University/Detroit Receiving Hospital
Coauthor(s): Jeffrey A Manko, MD, Assistant Professor of Clinical Surgery/Emergency Medicine, Director, Emergency Medicine Residency Program, Consulting Staff, Emergency Medicine Services, New York University/Bellevue Medical Center; Sally Santen, MD, Program Director, Assistant Professor, Department of Emergency Medicine, Vanderbilt University
Contributor Information and Disclosures

Updated: Dec 8, 2008

Introduction

Background

Acute cholangitis is a bacterial infection superimposed on an obstruction of the biliary tree most commonly from a gallstone, but it may be associated with neoplasm or stricture.

Pathophysiology

The main factors in the pathogenesis of acute cholangitis are biliary tract obstruction, elevated intraluminal pressure, and infection of bile. A biliary system that is colonized by bacteria but is unobstructed, typically does not result in cholangitis. It is believed that biliary obstruction diminishes host antibacterial defenses, causes immune dysfunction, and subsequently increases small bowel bacterial colonization. Although the exact mechanism is unclear, it is believed that bacteria gain access to the biliary tree by retrograde ascent from the duodenum or from portal venous blood. As a result, infection ascends into the hepatic ducts, causing serious infection. Increased biliary pressure pushes the infection into the biliary canaliculi, hepatic veins, and perihepatic lymphatics, leading to bacteremia (25-40%). The infection can be suppurative in the biliary tract.

The bile is normally sterile. In the presence of gallbladder or common duct stones (CBD), however, the incidence of bactibilia increases. The most common organisms isolated in bile are Escherichia coli (27%), Klebsiella species (16%), Enterococcus species (15%), Streptococcus species (8%), Enterobacter species (7%), and Pseudomonas aeruginosa (7%). Organisms isolated from blood cultures are similar to those found in the bile. The most common pathogens isolated in blood cultures are E coli (59%), Klebsiella species (16%), Pseudomonas aeruginosa (5%), and Enterococcus species (4%). In addition, polymicrobial infection is commonly found in bile cultures (30-87%) and less frequent in blood cultures (6-16%). For related pathophysiology, please see the Cholelithiasis and Cholecystitis and Biliary Colic articles.

Primary sclerosing cholangitis is a chronic liver disease that is thought to be due to an autoimmune mechanism. It is characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. This condition ultimately leads to portal hypertension and cirrhosis of the liver with the only definitive treatment being a liver transplant. For more on this condition, please refer to the Primary Sclerosing Cholangitis article.

Frequency

United States

Cholangitis is relatively uncommon. It occurs in association with other diseases that cause biliary obstruction and bactibilia (eg, after endoscopic retrograde cholangiopancreatography [ERCP], 1-3% of patients develop cholangitis). Risk is increased if dye is injected retrograde.

International

Recurrent pyogenic cholangitis, sometimes referred to as Oriental cholangiohepatitis, is endemic to Southeast Asia. It is characterized by multiple occurrences of biliary tract infection, intrahepatic and extrahepatic biliary stone formation, hepatic abscesses, and dilatation and stricturing of the intrahepatic and extrahepatic bile duct. For more on this condition, please refer to the Recurrent Pyogenic Cholangitis article.

Mortality/Morbidity

• Mortality of cholangitis is high due to the predisposition in people with underlying disease. Historically, the mortality rate was 100%. With the advent of endoscopic retrograde cholangiography, therapeutic endoscopic sphincterotomy, stone extraction, and biliary stenting, the mortality rate has significantly declined to approximately 5-10%.
• The following patient characteristics are associated with higher morbidity and mortality rates:
  • Hypotension
  • Acute renal failure
  • Liver abscess
  • Cirrhosis
  • Inflammatory bowel disease
  • High malignant strictures
  • Radiologic cholangitis – Post percutaneous transhepatic cholangiography
  • Female gender
  • Age older than 50 years
  • Failure to respond to antibiotics and conservative therapy
• Advanced age, concurrent medical problems, and delay in decompression increase the emergent operative mortality rate (17-40%).
• The mortality rate of elective surgery after medical stabilization is significantly less (approximately 3%).
• In the past, suppurative cholangitis was thought to have increased morbidity; however, prospective studies have not found this to be true.

Race

• Cholangitis frequently occurs secondary to a gallstone obstructing the common bile duct. Therefore, it carries the same risk factors as that of  cholelithiasis.
• Prevalence of gallstones is highest in fair-skinned people of Northern European descent as well as in Hispanic populations, Native Americans, and Pima Indians.
• In addition, certain Asian populations and inhabitants of countries where intestinal parasites are common are also at increased risk. Asians are more likely to have primary stones due to chronic biliary infections, parasites, bile stasis, and biliary strictures. Recurrent pyogenic cholangitis (Oriental cholangiohepatitis) rarely is observed in the United States
• African Americans with sickle cell disease are at increased risk.

Sex

• Although gallstones are more common in women than in men, the male-to-female ratio is equal in cholangitis.

Age

• Elderly patients are more likely to progress from asymptomatic gallstones to serious complications of gallstones and cholangitis.
• Suspect cholangitis in older patients presenting with sepsis and mental status changes. Elderly patients are more prone to gallstones and CBD stones and, therefore, cholangitis.
• The median age at presentation is between 50 and 60 years.

Clinical

History

In 1877, Charcot described cholangitis as a triad of findings of right upper quadrant (RUQ) pain, fever, and jaundice. The Reynolds pentad adds mental status changes and sepsis to the triad. A spectrum of cholangitis exists, ranging from mild symptoms to fulminant overwhelming sepsis. With septic shock, the diagnosis can be missed in up to 25% of patients. Consider cholangitis in any patient who appears septic, especially in patients who are elderly, jaundiced, or who have abdominal pain. A history of abdominal pain or symptoms of gallbladder colic may be a clue to the diagnosis.

• Charcot’s triad consists of fever, RUQ pain, and jaundice. It is reported in up to 50-70% of patients with cholangitis. However, recent studies believe it is more likely to be present in 15-20% of patients.
• Fever is present in approximately 90% of cases.
• Abdominal pain and jaundice is thought to occur in 70% and 60% of patients, respectively.
• Patients present with altered mental status 10-20% of the time and hypotension approximately 30% of the time. These signs, combined with Charcot’s triad, constitute Reynolds pentad.
• Consequently, many patients with ascending cholangitis do not present with the classic signs and symptoms.
• Most patients complain of RUQ pain; however, some patients (ie, elderly persons) are too ill to localize the source of infection.
• Other symptoms include the following:
  • Jaundice
  • Fever, chills, and rigors
  • Abdominal pain
  • Pruritus
  • Acholic or hypocholic stools
  • Malaise
• The patient's medical history may be helpful. For example, a history of the following increases the risk of cholangitis:
  • Gallstones, CBD stones
  • Recent cholecystectomy
  • Endoscopic manipulation or ERCP, cholangiogram
  • History of cholangitis
  • History of HIV or AIDS: AIDS-related cholangitis is characterized by extrahepatic biliary edema, ulceration, and obstruction. The etiology is uncertain, but it may be related to cytomegalovirus or Cryptosporidium infections. The management of this condition is described below, although decompression is usually not necessary.

Physical

• In general, patients with cholangitis are quite ill and frequently present in septic shock without an apparent source of the infection.
• Physical examination may reveal the following:
  • Fever (90%), although elderly patients may have no fever
  • RUQ tenderness (65%)
  • Mild hepatomegaly
  • Jaundice (60%)
  • Mental status changes (10-20%)
  • Sepsis
  • Hypotension (30%)
  • Tachycardia
  • Peritonitis (uncommon, and should lead to a search for an alternative diagnosis)

Causes

In Western countries, choledocholithiasis is the most common cause of acute cholangitis, followed by ERCP and tumors.

Any condition that leads to stasis or obstruction of bile in the CBD, including benign or malignant stricture, parasitic infection, or extrinsic compression by the pancreas, can result in bacterial infection and cholangitis. Partial obstruction is associated with a higher rate of infection than complete obstruction.

• CBD stones predispose patients to cholangitis.
  • Approximately 10-15% of patients with cholecystitis have CBD stones.
  • Approximately 1% of patients post cholecystectomy have retained CBD stones. Most CBD stones are immediately symptomatic, while some remain asymptomatic for years.
  • Some CBD stones are formed primarily rather than secondarily to gallstones.
• Obstructive tumors cause cholangitis. Partial obstruction is associated with an increased rate of infection compared with that of complete neoplastic obstruction.
  • Pancreatic cancer
  • Cholangiocarcinoma
  • Ampullary cancer
  • Porta hepatis tumors or metastasis
• Additional causes of cholangitis include the following:
  • Strictures or stenosis
  • Endoscopic manipulation of the CBD
  • Choledochocele
  • Sclerosing cholangitis (from biliary sclerosis)
  • AIDS cholangiopathy
  • Ascaris lumbricoides infections

• Search for CME/CE on This Topic »

• Recurrent Pyogenic Cholangitis (Gastroenterology)
• Primary Sclerosing Cholangitis (Pediatrics: General Medicine)
• Cholangitis, Primary Sclerosing (Radiology)
• Cholangitis, Recurrent Pyogenic (Radiology)

• Endoscopic and Surgical Management of Primary Sclerosing Cholangitis
• The Medical Management of Primary Sclerosing Cholangitis
• Review: Current Status of Therapy in Autoimmune Liver Disease