Presentation
Abdominal pain and jaundice.
Patient Data
There is intra and extrahepatic duct dilatation with an apparent caliber change in the mid-CBD as it passes through the pancreatic head.
The pancreas has lost its normal lobulation and appears generally swollen with some very mild peri-pancreatic fat standing. Of note the main pancreatic duct is not dilated and no focal pancreatic mass is identified.
Focal area of cortical hypoenhancement within the left kidney lower pole.
There is subtle but definite soft tissue cuffing around the abdominal aorta, infrarenal aorta and the common iliac arteries.
MRCP confirms a CBD stricture with abrupt tapering. No filling defect within the CBD to suggest stone disease.
MR also shows diffuse change in the pancreas which appears "sausage-like" with no pancreatic duct dilatation.
Interval CT shows the CBD stricture has been successfully stented with a plastic removable stent. The pancreatic changes have improved significantly.
There has been interval worsening of the foci of renal cortical hypo-enhancement which are now bilaterally increasing in size and number.
The para-aortic soft tissue cuffing appears fairly stable.
Case Discussion
This case illustrates a number of Imaging-specific features highlighted in the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) classification criteria for IgG4-related disease with the abdomen.
Pancreas and biliary tree
diffuse pancreatic enlargement (loss of lobulation) usually encompassing >2/3 of the pancreas
pancreas (either of the above) and biliary tree involvement (typically proximal biliary tract)
Renal
bilateral renal cortex low-density areas
Retroperitoneum
diffuse thickening of the abdominal aortic wall
circumferential or anterolateral soft tissue around the infrarenal aorta or iliac arteries
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