Differentials
Secondary sclerosing cholangitis
SIGNS / SYMPTOMS
History significant for a potential cause of the pathological process (e.g., intraductal stone disease, recurrent pyogenic cholangitis, recurrent pancreatitis, surgical trauma to the bile duct, bile duct ischaemia, caustic injury from intra-arterial chemotherapy, malignancy).[25]
No differentiating signs/symptoms.
INVESTIGATIONS
Investigations are targeted at the underlying cause. Identification of an underlying cause excludes primary sclerosing cholangitis.
Immunoglobulin G4 cholangitis
SIGNS / SYMPTOMS
Predominantly affects older men (average age 65 years), often with long-term exposure to potentially harmful chemicals.[2][49]
Obstructive jaundice is a more frequent presentation.
Not associated with inflammatory bowel disease.
INVESTIGATIONS
HISORt criteria (histological, imaging, and serological [IgG4] findings, other organ involvement, response to corticosteroid treatment).[2]
Cholangiography more frequently demonstrates stricture of the distal common bile duct and irregular narrowing of the main pancreatic duct - strictures are longer and band shaped.[2]
May be associated with autoimmune pancreatitis (elevated lipase and amylase, diffuse or localised enlargement of the pancreas on imaging).
IgG4 plasma cell infiltration in liver or duodenal tissue.
Dramatic response to corticosteroids.
Autoimmune hepatitis
SIGNS / SYMPTOMS
Female predominance.
INVESTIGATIONS
Higher titres of antinuclear antibodies (ANA) and/or smooth muscle antibodies.[7][50]
Elevated levels of serum immunoglobulin G (IgG).
More marked aminotransferase elevations and less prominent alkaline phosphatase elevations.
Normal cholangiogram.
Liver biopsy with interface hepatitis, plasma cell infiltrate, bridging necrosis.
Biochemical improvement with corticosteroid treatment in most cases.
Diagnostic criteria published by the International Autoimmune Hepatitis Group.[50]
Primary biliary cholangitis
SIGNS / SYMPTOMS
Predominantly affects middle-aged women.
Not associated with inflammatory bowel disease.
INVESTIGATIONS
Antimitochondrial antibody present in 95% of cases.[51]
Elevated levels of serum immunoglobulin M (IgM).
Normal cholangiogram (although decreased branching and narrowing of the intrahepatic ducts can be seen in patients with cirrhosis of any cause, and can be difficult to distinguish from intrahepatic PSC).[43]
Use of this content is subject to our disclaimer