A. Types of Cholangitis [5]

1. Bacterial (Ascending) Cholangitis
   1. Choledocholithiasis (obstructive) is most common cause (~90%)
   2. Obstruction of ampulla of Vater or common bile duct also occurs
   3. Malignant obstruction of biliary tree
   4. Biliary fistula
   5. Recurrent pyogenic cholangitis
   6. Caroli's Disease - congenital dilation of large hepatic ducts, very rare
2. Sclerosing Cholangitis - likely autoimmune

B. Pathogenesis

1. Bacterial invasion of biliary tree
2. Usually ascending infection derived from small intestine
3. Nearly always in setting of gallstone lodging in biliary tree (common bile duct)
4. Common Organisms
   1. ~90% Gram Negative Enteric Rods (majority are E. coli)
   2. ~5% of cases with Enterococcus
   3. Concern for gas forming organisms (Clostridia) and other anaerobes
5. Diabetics at increased risk for cholangitis, and often have mixed infections
6. May cause marked hepatitis, jaundice, severe liver inflammation
7. May progress to sepsis syndrome unless treated aggressively
8. Occasionally caused by helminths such as ascariasis, liver fluke [6]

C. Symptoms of Bacterial Cholangitis

1. Charcot's Triad
   1. Right upper quadrant abdominal pain
   2. Fever/chills
   3. Jaundice
2. Nausea and Vomiting
3. Dehydration (third spacing of fluid)
4. Severe acute cholangitis can have >25% mortality risk

D. Diagnosis [2]

1. Ultrasound is insensitive for diagnosis common duct stones
2. Conventional or helical (spiral) CT scan is more sensitive than ultrasound
3. Endoscopic retrograde cholangeopancreotography (ERCP) [3]
   1. Best for diagnostic confirmation
   2. May also be used for emergent or elective removal of stones

E. Differential Diagnosis [2]

1. Cholecystitis
   1. May be a precipitant and/or coexist with bacterial cholangitis
   2. Most often appears to precede ascending cholangitis
2. Pancreatitis
3. Duodenitis
4. Peptic Ulcer Disease
5. Renal Stone

F. Treatment

1. Supportive therapy, including IV fluids, vasopressors as needed, no oral intake
2. Antibiotics
   1. Mild Disease: Cefotetan or Cefoxitin or Mezlocillin or Piperacillin
   2. Moderate to Severe: ampicillin or vancomycin / gentamicin / metronidazole
   3. Ceftriaxone should be avoided as this can worsen sludge and stones
   4. Aztreonam or ceftazidime may be substituted for gentamicin
3. Invasive Therapy
   1. ERCP versus surgical intervention if condition not stabilized
   2. For choledocholithiasis (common bile duct stone), ERCP is prefferred treatment
   3. Endoscopic biliary drainage is safer and more effective than surgery [4]
   4. Significant reduction in mortality due to ERCP instead of open surgery
   5. Stone is removed and sphincterotomy is performed
4. ERCP can be used to guide diagnosis, anatomy, of periampullary tumors, etc.
5. ERCP for acute, rapid, opening of biliary tree

References

1. Lai ECS, Mok FPT, Tan ESY, et al. 1992. NEJM. 326:1582
2. Saini S. 1997. NEJM. 336(26):1889
3. Brugge WR and Van Dam J. 1999. NEJM. 341(24):1808
4. Leung JWC, Chung SCS, Sung JJY, et al. 1989. Lancet. 1:1307
5. Chung RT and Sheffer EC. 2001. NEJM. 345(11):817 (Case Record)
6. Hurtado RM, Sahani DV, Kradin RL. 2006. NEJM. 354(12):1295 (Case Record)