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.1 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.2 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.3 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.4
- 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
- Cholangiocarcinoma5
- 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
More on Cholangitis |
Overview: Cholangitis |
| Differential Diagnoses & Workup: Cholangitis |
| Treatment & Medication: Cholangitis |
| Follow-up: Cholangitis |
| Multimedia: Cholangitis |
| References |
| Further Reading |
| Next Page » |
References
Aron JH, Bowlus CL. The immunobiology of primary sclerosing cholangitis. Semin Immunopathol. May 26 2009;[Medline].
Kashyap R, Mantry P, Sharma R, Maloo MK, Safadjou S, Qi Y, et al. Comparative Analysis of Outcomes in Living and Deceased Donor Liver Transplants for Primary Sclerosing Cholangitis. J Gastrointest Surg. May 9 2009;[Medline].
van Erpecum KJ. Gallstone disease. Complications of bile-duct stones: Acute cholangitis and pancreatitis. Best Pract Res Clin Gastroenterol. 2006;20(6):1139-52. [Medline].
Kinney TP. Management of ascending cholangitis. Gastrointest Endosc Clin N Am. Apr 2007;17(2):289-306, vi. [Medline].
Jabara B, Fargen KM, Beech S, Slakey DR. Diagnosis of cholangiocarcinoma: a case series and literature review. J La State Med Soc. Mar-Apr 2009;161(2):89-94. [Medline].
Rustemovic N, Cukovic-Cavka S, Opacic M, Petrovecki M, Hrstic I, Radic D, et al. Endoscopic ultrasound elastography as a method for screening the patients with suspected primary sclerosing cholangitis. Eur J Gastroenterol Hepatol. Jun 2 2009;[Medline].
Itoi T, Kawai T, Sofuni A, Itokawa F, Tsuchiya T, Kurihara T, et al. Efficacy and safety of 1-step transnasal endoscopic nasobiliary drainage for the treatment of acute cholangitis in patients with previous endoscopic sphincterotomy (with videos). Gastrointest Endosc. Jul 2008;68(1):84-90. [Medline].
Rosing DK, De Virgilio C, Nguyen AT, et al. Cholangitis: analysis of admission prognostic indicators and outcomes. Am Surg. Oct 2007;73(10):949-54. [Medline].
Bornman PC, van Beljon JI, Krige JE. Management of cholangitis. J Hepatobiliary Pancreat Surg. 2003;10(6):406-14. [Medline].
Hanau LH, Steigbigel NH. Acute (ascending) cholangitis. Infect Dis Clin North Am. Sep 2000;14(3):521-46. [Medline].
Jain MK, Jain R. Acute bacterial cholangitis. Curr Treat Options Gastroenterol. Apr 2006;9(2):113-21. [Medline].
Lai EC. Management of severe acute cholangitis. Br J Surg. Jun 1990;77(6):604-5. [Medline].
Lipsett PA, Pitt HA. Acute cholangitis. Surg Clin North Am. Dec 1990;70(6):1297-312. [Medline].
Muir CA. Acute ascending cholangitis. Clin J Oncol Nurs. Apr 2004;8(2):157-60. [Medline].
Qureshi WA. Approach to the patient who has suspected acute bacterial cholangitis. Gastroenterol Clin North Am. Jun 2006;35(2):409-23. [Medline].
Romagnuolo J, Bardou M, Rahme E, et al. Magnetic resonance cholangiopancreatography: a meta-analysis of test performance in suspected biliary disease. Ann Intern Med. Oct 7 2003;139(7):547-57.
Sievert W, Vakil NB. Emergencies of the biliary tract. Gastroenterol Clin North Am. Jun 1988;17(2):245-64. [Medline].
Sinanan MN. Acute cholangitis. Infect Dis Clin North Am. Sep 1992;6(3):571-99. [Medline].
van den Hazel SJ, Speelman P, Tytgat GN, et al. Role of antibiotics in the treatment and prevention of acute and recurrent cholangitis. Clin Infect Dis. Aug 1994;19(2):279-86. [Medline].
Yusoff IF, Barkun JS, Barkun AN. Diagnosis and management of cholecystitis and cholangitis. Gastroenterol Clin North Am. Dec 2003;32(4):1145-68. [Medline].
Further Reading
Clinical guidelines
ACR Appropriateness Criteria® right upper quadrant pain.
American College of Radiology - Medical Specialty Society. 1996 (revised 2005). 5 pages. [NGC Update Pending] NGC:004781
AASLD practice guidelines: evaluation of the patient for liver transplantation.
American Association for the Study of Liver Diseases - Private Nonprofit Research Organization. 2000 Jan (revised 2005 Jun). 26 pages. NGC:004333
Clinical trials
Compare Conventional Colonosocpy to Endoscopic AFI, NBI for Dysplasia Detection for Ulcerative Colitis & Cholangitis
Probiotics in Patients With Primary Sclerosing Cholangitis
Differential Gene Expression of Liver Tissue and Blood From Individuals With Chronic Viral Hepatitis
Related eMedicine topics
Cholangitis (Emergency Medicine) Cholangitis, Primary Sclerosing (Radiology)
Primary Sclerosing Cholangitis (Gastroenterology)
Primary Sclerosing Cholangitis (Pediatrics: General Medicine)
Keywords
cholangitis, gallstone, gall stone, gallbladder, biliary tract obstruction, common bile duct obstruction, primary sclerosing cholangitis, cholecystitis, biliary colic, cholelithiasis, cholangitis treatment, cholangitis symptoms, CBD, CBD stones
Overview: Cholangitis