IgG4-related sclerosing cholangitis

Case contributed by Dr Michael P Hartung

Presentation

Painless jaundice with negative endoscopic biopsies. Evaluate for tumor.

Patient Data

Age: 65 years
Gender: Male
Fluoroscopy

Baseline ERCP

ERCP images with multiple long-segment intrahepatic biliary ductal strictures. Multiple additional areas of ductal irregularity. A plastic common bile duct stent was placed just beyond the hilar stricture on the last image.

The main pancreatic duct is perhaps mildly irregular in the pancreatic head. 

MRI

Baseline MRI

Plastic common bile duct stent is present. 

Circular symmetric rind of enhancing tissue encasing the common bile duct and central hepatic ducts. 

Multifocal intrahepatic biliary ductal irregularity and stricturing. This can be well seen on the T2 fat sat and post contrast images (portal venous, delayed, and 20 minute). 

Diminished excretion of hepatobiliary contrast agent (Eovist) in the dilated segment 4 bile ducts on 20 minute delayed images. Several other areas of ductal narrowing/irregularity are present. 

Subtle focal enlargement of the pancreatic tail with mild T2 hyperintensity with hyperenhancement.

Fluoroscopy

2 months post-treatment ERCP

Plastic CBD stent has been removed. 

Dramatic improvement in multifocal biliary strictures compared to the baseline examination, with minor areas of residual irregularity. 

MRI

2 months post-treatment MRI

Note that several sequences were motion degraded and excluded from this study (including arterial and portal venous phases). 

Intrahepatic biliary ductal dilation is dramatically improved. This is particularly well seen when comparing the Axial T2 Fat Sat images. 

Decreased soft tissue thickening and enhancement of the common bile and central hepatic ducts. This can be appreciated on the Axial T2 fat sat and post-contrast images. Some mild ductal narrowing and irregularity persists, best appreciated on the hepatobiliary/excretory phase images. 

Persistent subtle enhancement and thickening of the pancreatic tail.

Case Discussion

IgG4 levels were greater than 300. Salivary gland biopsy demonstrated extensive infiltration of IgG4-positive plasma cells, which is a common additional site of involvement. 

This patient has a combination of IgG4-related sclerosing cholangitis and suspected focal autoimmune pancreatitis (AIP), with dramatic improvement after treatment with steroids. 

IgG4-positive plasma cells infiltrate the intra and extrahepatic bile ducts segments causing wall thickening and enhancement, and can result in extensive fibrosis. 

Imaging features can overlap with primary sclerosing cholangitis (PSC) and cholangiocarcinoma. The ERCP images provide some helpful features more typical of IgG4 such as longer, continuous segments of narrowing due to periductal fibrosis.

PSC tends to narrow the ducts in shorter segments ("beaded") and has an important association with inflammatory bowel disease. It does not respond to steroids. 

While a hilar cholangiocarcinoma could cause the central stricture on the initial ERCP, it would not cause multifocal narrowing seen elsewhere in the biliary tree. 

There is a strong association with AIP which is likely present focally in the pancreatic tail, which appears to be slower to resolve following steroids. 

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Case information

rID: 71113
Published: 20th Sep 2019
Last edited: 20th Sep 2019
Inclusion in quiz mode: Included

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