Epidemiology^[3]

Cholangitis is relatively uncommon in Europe and the US. 1-3% of patients develop it after ERCP. Its racial distribution pattern follows to some extent the races in which there is a high prevalence of gall stones - ie fair-skinned people of Northern European descent, Hispanics, native Americans and Pima Indians. There is also a subset of Asian patients who develop cholangitis, mainly secondary to parasitic infection (recurrent pyogenic cholecystitis).^[4] The male to female ratio is equal. The median age of presentation is 50-60.

Aetiology^[3]

• Obstruction of the gall bladder or bile duct due to stones
• ERCP
• Tumours - pancreatic cancer,cholangiocarcinoma,ampullary cancer,porta hepatis tumors or metastasis
• Bile duct stricture or stenosis
• Choledochocele (cyst or diverticulum of the CBD)
• AIDS cholangiopathy
• Parasitic infection - roundworm, liver fluke

Presentation

History^[3]

50-70% of patients present with the classic Charcot triad of jaundice, fever and right upper quadrant (RUQ) pain. In elderly patients, the abdominal pain may be poorly localised. 10-20% of patients also present with the additional features of hypotension due to septic shock and mental confusion - the Reynolds pentad. The patient may also report acholic (putty-coloured) stools and pruritus. A history of gallstones, CBD stones, recent cholecystectomy, ERCP or other invasive procedures, HIV or AIDS may assist the diagnosis. Some patients present with several attacks, usually in association with untreated biliary stones (recurrent pyogenic cholangitis).

Examination

Physical signs may include fever, RUQ tenderness, jaundice, mental status changes, hypotension and tachycardia. Peritonism is an unusual sign and should stimulate the search for an alternative diagnosis.

Differential diagnosis

• Cholecystitis and biliary colic
• Diverticular disease
• Hepatitis
• Mesenteric ischaemia
• Pancreatitis
• Other causes of septic shock
• Cirrhosis
• Liver failure
• Liver abscess
• Acute appendicitis
• Perforated peptic ulcer
• Pyelonephrosis
• Right colon diverticulitis

Investigations

Laboratory tests^[3]

• The white cell count is raised in the majority of cases.
• Blood cultures are positive in between 20-30% of cases.
• Liver function tests are likely to show hyperbilirubinaemia, increased alkaline phosphatase and mildly raised aspartate aminotransferase and alanine aminotransferase levels.
• One third of patients have a raised amylase. This often indicates involvement of the lower part of the CBD.
• If bile fluid is available (eg biliary drainage through intervention has occurred), send a sample off for culture.

Imaging^[3]

• Abdominal Plain X-ray is the least invasive method, but is generally unhelpful unless the patient has ileus or has air in the biliary tree, which may be seen after endoscopic manipulation, emphysematous cholecystitis, cholangitis or cholecystic-enteric fistula. A ring of calcium is occasionally seen around gallstones (10-30%).
• Ultrasound may be useful in identifying gallstones, but is less successful in identifying stones in the CBD. It is however, of value in identifying a dilated duct, signifying obstruction. Ultrasound is readily available and useful in identifying complications such as empyema. It is however highly dependent on operator skill and the cystic duct cannot be imaged. A normal scan does not rule out acute cholangitis.
• CT scanning may well replace ultrasound as the investigation of choice and produces excellent images of the biliary tree, especially when spiral^[5] or helical^[6] methods are used. Other pathologies such as ampullary tumours can be viewed and it is also useful in excluding differential diagnoses such as right-sided diverticulitis and ruptured appendix.
• ERCP is useful in patients in who need intervention as well as imaging. It has a major complication rate of 5.4% and a mortality rate of 0.49% as should not therefore be used solely for diagnostic purposes.^[3]
• Magnetic resonance cholangiopancreatography (MRCP) is as sensitive as ERCP and is a noninvasive technique particularly useful if biliary stones are suspected.^[7][8] Contraindications are as for MRI (eg presence of a cardiac pacemaker, cerebral aneurysm clips, ocular or cochlear implants and ocular foreign bodies) and it is, in contrast to ERCP, purely a diagnostic rather than therapeutic tool.
• Biliary scintigraphy uses a radio-active substance which is excreted through the biliary system and can be used in cases of diagnostic doubt to demonstrate obstruction of the CBD. Positive changes (failure of the substance to enter the intestinal tract) may be detected before duct enlargement can be demonstrated on ultrasound. It is less sensitive in patients with high bilirubin levels.^[9]

Management

• Resuscitation may be required for patients with septic shock and due attention should be given to oxygenation and correction of fluid and electrolyte imbalance.
• Vital signs should be monitored.
• Parenteral antibiotics should be administered once blood cultures have been taken.The drugs selected should be effective against anaerobes and Gram negative organisms. These may include ampicillin, piperacillin and gentamicin.^[3]
• Patients who fail to respond to 24-48 hours of antibiotics, or those with a rapidly deteriorating clinical picture, will require urgent biliary decompression:
  • Endoscopic decompression with removal of any associated stones is the usual initial procedure. If large stones and/or a considerable degree of sepsis is present, a biliary stent may be employed either as an adjunct to or as a replacement for endoscopic decompression.
  • Transhepatic biliary drainage is reserved for endoscopic failures and surgical biliary decompression via open laparotomy is rarely required.
  • Further surgery may be required electively once the patient stabilises, if the initial cause has not been dealt with at the time of emergency surgery.^[10]
  • Recurrent pyogenic cholangitis may require more radical surgery such as liver resection.^[11]
• The international symposium on acute cholangitis recommended the following:^[12]
  • Stage I - observation
  • Stage II - early biliary drainage
  • Stage III - urgent biliary drainage

At any stage, the underlying aetiology needs treatment (eg endoscopic treatment and surgery).

Complications^[3]

Patients with severe sepsis can develop liver abscesses and liver failure, bacteraemia and Gram-negative sepsis. Acute renal failure can also ensue. Patients treated with percutaneous or endoscopic drainage can develop intra-abdominal or percutaneous bleeding, sepsis, fistulae and bile leakage.

Prognosis^[3]

The current mortality figures are 7-40%. A poor prognosis is associated with:

• 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
• A white cell count on admission of ≥20 x 10⁹/L and total bilirubin of ≥880 μmol/L (10mg/dL)^[13]