Cholangitis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2],Farwa Haideri [3]
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Overview
Cholangitis is an infection of the bile duct, which transports bile from the liver to the intestines and the gallbladder. Symptoms include fever, right upper quadrant pain, and jaundice due to the infection of the bile duct and inflammation of the biliary tree, which is usually the result of obstruction and stasis.
Historical Perspective
- Dr. Jean-Martin Charcot, a French physician, is credited with discovering the disease in the late 19th century. He referred to the condition as "hepatic fever."
- Charcot's triad of fever, jaundice, and right upper quadrant abdominal pain is the classical presentation of cholangitis.
- By adding septic shock and mental status changes to the list of symptoms, Dr. B. M. Reynolds and Dr. Everett L. Dargan changed Charcot's triad to Reynold's pentad.
- Until 1968, the mainstay of treatment of cholangitis was surgery, with exploration of the bile duct and excision of gallstones, until the advent of endoscopic retrograde cholangiopancreatography (ERCP).
Classification
Acute cholangitis is classified into grade I, II, or III, depending on the severity of the condition.
Pathophysiology
Cholangitis involves two main factors: an increase in the bacterial presence and elevated intraductal pressure in the bile duct, both of which allow for the translocation of bacteria or endotoxins in the vascular system. Bacterial contamination alone does not usually result in cholangitis. Increased pressure in the biliary system, from obstruction in the bile duct, widens the spaces between the cells lining the duct, which brings bacterially contaminated bile into the bloodstream.
Causes
Cholangitis is usually caused by a bacterial infection, which can occur due to blockage in the duct, such as from a gallstone or tumor. The infection causing this condition may also spread to the liver.
Differential Diagnosis
Cholangitis must be differentiated from other causes of infection in the common bile duct, as well as from inflammation and infection of cholecystitis.
Epidemiology and Demographics
Cholangitis is most prevalent in adults, with roughly 20% of the adult population suffering from some form of abdominal pain from gallstones passing through the bile duct into the digestive tract.
Risk Factors
Common risk factors in the development of cholangitis are gallstones, sclerosing cholangitis, and HIV. Variations in treatment and risk factors influence mortality rates in patients with cholangitis, and these rates underscore the necessity for standardized diagnostic, treatment, and severity assessment criteria.
Screening
There are no established screening processes for cholangitis or cholangiocarcinoma, a cancer associated with this disease. There are methods to detect the early onset of both diseases.
Natural History, Complications, and Prognosis
Patients who show early signs of multiple organ failure (renal failure, disseminated intravascular coagulation, alterations in the level of consciousness, and shock) or evidence of acute cholangitis, as well as those who do not respond to conservative treatment, should receive systemic antibiotics and undergo emergent biliary drainage. Unless early and appropriate biliary drainage is performed and systemic antibiotics are administered, death will occur. Prognosis is usually good with treatment, but poor without it.
Diagnosis
History and Symptoms
A positive history of gallstones and common bile duct stones, recent cholecystectomy, endoscopic manipulation or endoscopic retrograde cholangiopancreatography (ERCP), cholangiogram and history of HIV or AIDS. Symptoms of cholangitis include fever, abdominal pain, nausea and vomiting, jaundice/yellowish discoloration of skin, acholic stools/pale stools, pruritus, malaise, and confusion
Physical Examination
Charcot's triad, which includes abdominal pain, jaundice, and fever, describes three common findings in cholangitis. Reynold's pentad, which includes Charcot's triad and two other symptoms, septic shock and mental confusion, also provides common markers in a physical examination for cholangitis. Cholangitis is associated with significant morbidity and mortality.
Laboratory Findings
Laboratory tests provide useful clues in the diagnosis of cholangitis. Some commonly conducted tests include complete blood count, basic metabolic panel, liver function tests, blood culture, and other body fluid cultures.
X-Ray
There are no x-ray findings associated with acute cholangitis.
CT
CT scans may be helpful in locating, with high sensitivity, the site of the obstruction responsible for a case of cholangitis.
MRI
There are no MRI findings associated with acute cholangitis.
Ultrasound
Ultrasounds (US) are the primary imaging modality for cholangitis. An US is both sensitive and specific in demonstrating biliary dilatation.
Other Imaging Findings
There are no other imaging findings associated with cholangitis
Other Diagnostic Studies
Endoscopic retrograde cholangiopancreatography (ERCP) is considered a gold standard test for biliary obstruction. Magnetic resonance cholangiopancreatography (MRCP) and Percutaneous transhepatic cholangiography (PTCA) are the most sensitive techniques to correctly determine the underlying cause and level of biliary obstruction in patients with acute cholangitis when ERCP fails.
Diagnostic Criteria
Shown below are the diagnostic criteria for acute cholangitis according to Tokyo guidelines:
- The diagnosis is "suspected" in the case of the presence of one item in systemic inflammation with one item in either cholestasis or imaging findings.
- The diagnosis is "definite" in the case of the presence of one item in systemic inflammation, one item in cholestasis, and one item in imaging.
| Clinical Manifestations | Changes from the baseline |
|---|---|
| Systemic inflammation | ♦ Fever >38℃ and/or shaking chills ♦ Evidence of inflammatory response: - WBC (×1000/μl) <4, or >10 - CRP (mg/dl) ≥1 |
| Cholestasis | ♦ Jaundice with total bilirubin ≥2 (g/dl) ♦ Abnormal liver function tests: - ALP (IU) >1.5×STD - γGTP (IU) >1.5×STD - AST (IU) >1.5×STD - ALT (IU) >1.5×STD |
| Imaging findings | ♦ Biliary dilatation ♦ Evidence of the etiology (stricture, stone, stent, etc.) on imaging (abdominal X-ray: KUB, abdominal USG, CT scan, MRI, MRCP, and HIDA scan) |
Severity Assessment Criteria
The severity assessment criteria for acute cholangitis according to Tokyo guidelines are as follows:
Grade III Acute Cholangitis
Grade III, or severe acute cholangitis, is characterized by the onset of dysfunction in at least one of the following:
- Cardiovascular system: decreased blood pressure that necessitates the administration of dopamine (>5 μg/kg/min) or norepinephrine
- Neurological system: abnormal consciousness
- Respiratory system: PaO2/FiO2 ratio <300
- Renal system: serum creatinine >2.0 mg/dl, decreased urine output
- Hepatic system: PT-INR >1.5
- Hematological system: platelet count < 100,000/mm3
Grade II Acute Cholangitis
Grade II, or moderate acute cholangitis, is characterized by the presence of any two of the following:
- Abnormal WBC count: >12,000/mm3, <4,000/mm3
- Fever ≥39°C
- Age ≥75 years
- Elevated total bilirubin ≥5 mg/dl
- Decreased albumin level <0.7 x STD
Grade I Acute Cholangitis
Grade I, or mild acute cholangitis, does not meet the criteria of either grade II (moderate) or grade III (severe) acute cholangitis.
Treatment
Medical Therapy
Antimicrobial therapy is indicated for acute cholangitis. Patients with community-acquired mild-to-moderate disease are treated with Cephalosporins. All other patients are treated with a combination of Metronidazole and either Imipenem-Cilastatin, Meropenem, Doripenem, Piperacillin-Tazobactam, Ciprofloxacin, Levofloxacin, or Cefepime.
Surgery
Surgery is not recommended for the treatment of cholangitis.
Primary Prevention
Although re-establishing biliary drainage is the main focus of treatment, antibiotics play an important role in the management of cholangitis.
Secondary Prevention
Secondary prevention strategies for cholangitis include continued treatment of predisposing causes in appropriate patients.
Cost-Effectiveness of Therapy
The most cost-effective technique to diagnose cholangitis is an ultrasound.