IgG4 disease - autoimmune pancreatitis with sclerosing cholangitis

Case contributed by Dr Jake Ball

Presentation

Painless jaundice.

Patient Data

Age: 85 years
Gender: Male

The CBD is dilated with associated intrahepatic duct dilatation, particularly within the left lobe. There is abrupt cut-off of the CBD caliber at the level of the pancreatic head. There is periductal thickening and enhancement at this level which was obscured on the arterial phase imaging by motion artefact.

Further narrowing and periductal enhancement are noted at the porta hepatis. A third intrahepatic area of narrowing is seen further distally in the left liver. These 3 areas of stricturing and entire pancreas demonstrate diffusion restriction.

The pancreas appears bulky in appearance, with increased diffusion restriction.
No pancreatic duct dilatation. No focal pancreatic lesion is
identified. No peripancreatic free fluid is present.

Case Discussion

This patient had ERCP and biopsy of the CBD stricture with no malignant cells identified. Immunological serology demonstrated elevated IgG4. 

The main differential to exclude in a patient of this age is cholangiocarcinoma. The associated diffusion restriction of the pancreas and elevated IgG4 with a negative biopsy makes the diagnosis of autoimmune pancreatitis with associated IgG4-related sclerosing cholangitis the favored differential. These entities are associated 90% of the time.

The patient's age and absence of inflammatory bowel disease make primary sclerosing cholangitis less likely, which although also autoimmune related, is a separate entity.

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