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- Acute infection due to partial or complete obstruction of the biliary tree, most commonly by gallstones migrating into the common bile duct (CBD); also by endoscopic retrograde cholangiopancreatography (ERCP), tumor, and stricture
- Primary choledocholithiasis (formation of stones in the CBD) is uncommon; occurs with bile stasis (e.g., cystic fibrosis, distal biliary stricture)
- Must distinguish from primary sclerosing cholangitis (PSC), a chronic progressive disorder of unknown etiology characterized by inflammation, fibrosis, and strictures with intra- and/or extrahepatic bile ducts
- Severity ranges from mild pain and low-grade fever to life-threatening sepsis.
- When suspected, urgent intervention is needed.
- System(s) affected: GI tract, hepatobiliary, other systems via hematogenous spread
Parallels the prevalence of gallstones; stones migrating into the CBD are associated with 90% of cases. Median age of presentation is between 50 and 60 years; incidence increases with age.
- Stones found most commonly in people of Northern European, Hispanic, and Native American descent
- Rare in children (except in hemolytic disorders such as sickle cell disease)
- CBD stones are discovered incidentally in 5–20% of patients undergoing routine right upper quadrant (RUQ) evaluation for gallstones.
- Acute cholangitis occurs in 6–9% of patients hospitalized with gallstone disease.
- No gender differences have been reported.
- Patients with CBD stones have positive blood cultures in 20–30% of cases.
Etiology and Pathophysiology
- Obstruction of biliary flow by stones (90% of cases), neoplasms, or strictures promotes bile stasis and spread of bacteria from biliary tree into hepatic ducts.
- Increased intraluminal pressure decreases intrabiliary IgA secretion, disrupts hepatocellular tight junctions, and pushes bacteria into hepatic veins, biliary canaliculi, and perihepatic lymphatics, leading to bacteremia (25–40%). Bacteria gain access to the biliary tree via retrograde ascent from the duodenum.
- Rarely, infection enters from portal venous system, periportal lymphatics, an eroding hepatic abscess, or infected pancreatic fluid collection.
- The CBD may not be dilated if obstruction is partial, in which case an isolated alkaline phosphatase elevation is sometimes present.
- Pyogenic cholangitis in Asia is most commonly due to parasitic infection (Clonorchis sinensis, Opisthorchis viverrini, Ascaris lumbricoides, Schistosoma mansoni) and is characterized by intrahepatic stones and recurrent attacks.
- Most common bacterial organisms: Escherichia coli, Klebsiella pneumonia, and Enterococcus (1)[A]
- Others: Bacteroides fragilis, Streptococcus faecalis, Enterobacter, and Pseudomonas
- Anaerobes, including Clostridium and Bacteroides, are more frequent in polymicrobial infections and patients with prior biliary-enteric surgery.
- With biliary endoprosthesis, Pseudomonas and Enterococcal species more common
- Hospitalized patients are prone to methicillin-resistant Staphylococcus aureus, Pseudomonas species, and vancomycin-resistant Enterococcus.
- Cytomegalovirus, Cryptosporidium, Mycobacterium avium-intracellulare, and herpes simplex virus are common in HIV-related cholangiopathy. In HIV patients, acute cholecystitis may be acalculous due to infection, inflammation, or gallbladder wall ischemia.
Increased risk with family history of gallstones
- Cholelithiasis, CBD stones
- Chronic bile duct inflammation due to sclerosing cholangitis, infection, and possibly hypothyroidism
- Periampullary malignancy
- Advanced age >70 years, usually >50 years old
- Ileal Crohn disease
- Hepatobiliary infections
- Conditions predisposing to biliary stasis (diabetes mellitus, obesity, pregnancy, rapid weight loss, prolonged fasting)
- Hemolytic syndromes or biliary anomalies (children)
- Roux-en-Y bypass surgery
- Biliary strictures or neoplasms, including masses that compress the duct; chronic pancreatitis with inflammatory CBD stricture
- Endoscopic or surgical manipulation; biliary stent occurs in 10% postbiliary tract reconstruction.
- Medications: octreotide, ceftriaxone, estrogen
- HIV or AIDS
Gallstone risk increases during pregnancy due to biliary cholesterol supersaturation and decreased GB motility.
- Adequate physical activity
- Avoid foods rich in saturated fats.
- In patients with increased risk of biliary stasis: Rapid weight loss, long-term somatostatin therapy or parenteral nutrition, and use of ursodeoxycholic acid reduces lithogenicity.
- Long-term statin use reduces biliary cholesterol concentration of bile and lowers risk of gallstone formation (not recommended for routine prevention).
- Ensure patency of biliary tree with intraoperative cholangiography (IOC) at time of cholecystectomy; if operative view is not possible and CBD stone is suspected, endoscopic cholangiogram is recommended.
- Prophylactic antibiotics before ERCP
Commonly Associated Conditions
- Pancreatitis, acute cholecystitis
- Crohn disease