IgG4-related autoimmune pancreatitis and cholangitis

Case contributed by Michael P Hartung
Diagnosis certain

Presentation

Abdominal pain with recurrent pancreatitis. Evaluate for complications.

Patient Data

Age: 65 years
Gender: Male

Diffuse enlargement of the pancreas with minimal surrounding haziness/fluid. Perhaps some low-attenuation "halo" near the tail.

Concentric thickening and enhancement of the common bile duct. 

Case Discussion

Serum IgG4 levels (checked after interpreting radiologist raised concern of IgG4-related disease) = 1000 mg/dL (normal 8-140 mg/dL). Range of IgG4 values in AIP = 1234 +/-13471.

What findings should make you think about autoimmune/IgG4-related pancreatitis?

  • diffuse pancreatic enlargement
  • minimal peripancreatic inflammation/fluid
  • concentric thickening and enhancement of the common bile duct, indicating autoimmune cholangitis

Timely diagnosis is of great benefit to patients, as they can usually be managed with steroids alone. 

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