Introduction

• A group of inflammatory liver diseases with a spectrum of histological classifications including neutrophilic (suppurative), and inflammatory (lymphocytic and biliary cirrhosis)*.
• Cause: unknown, ascending infection from gut, immune-mediated inflammation.
• Signs: hepatic disease - icterus, pyrexia, anorexia, ascites, vomiting and diarrhea, lethargy, weight loss.
• Diagnosis: biochemistry, imaging, biopsy.
• Treatment: supportive as for hepatic disease, specific therapy depends on histological type.
• See also acute hepatic disease Liver: acute disease.
• * From the WSAVA Liver Standardization Group (2006):
  • Neutrophilic cholangitis (acute and chronic).
  • Lymphocytic cholangitis (acute and chronic).
  • Chronic cholangitis (fluke infestation in endemic areas (not UK)).
  • For neutrophilic and lymphocytic cholangitis terminology is descriptive of the major infiltrating cell type but mixed inflammatory reactions can occur. These are the two most clearly recognizable forms and both correlate to distinct, different clinical presentations that require different management.

Pathogenesis

Etiology

• Neutrophilic cholangitis: bacterial infection.
• Escherichia coli Escherichia coli, Clostridium spp Clostridia spp, Enterococcus spp, Bacteroides spp Bacteroides fragilis, Actinomyces spp Actinomyces pyogenes and Streptococcus spp Streptococcus spp have been isolated from biopsy specimens and more reliably from bile culture.
• Lymphocytic cholangitis: thought to be immune-mediated.

Specific

• Bile duct/ gall bladder anomalies Bile duct: disease Gallbladder disease: overview.
• Cholelithiasis.

Pathophysiology

Neutrophilic cholangitis/cholangiohepatitis (NC) (suppurative cholangiohepatitis)

• Ascending bacterial infection, biliary obstruction and bacterial translocation.
• Neutrophilic inflammation of bile duct which can extend beyond the limiting plate and into hepatic parenchyma. 
• Role of concurrent cholelithiasis, bile duct/gall bladder anomalies, pancreatitis and inflammatory enteropathy in etiopathogenesis is by association but mechanisms are unclear.
• Commonly occurs with pancreatitis Pancreatitis/intestinal inflammation (triaditis).
• Common pancreaticobiliary duct may contribute to risk of infection/inflammation.
• Biliary obstruction (extrahepatic bile duct obstruction; EHBDO) can occur with NC.

Lymphocytic cholangitis/cholangiohepatitis (LC) (non-suppurative cholangiohepatitis)

• Immune-mediated inflamation with unknown trigger.
• Infiltrate of small lymphocytes in portal region with varying degrees of fibrosis and bile duct proliferation.
• Can occur with inflammatory enteropathy and pancreatitis (triaditis) Pancreatitis.

Diagnosis

Treatment

Outcomes