A 71-year-old male with suspected pancreatic cancer was diagnosed with IgG4-related disease of the pancreas and biliary tree through histology showing lymphoplasmacytic inflammation, obliterative phlebitis, and elevated serum IgG4 (8.3 g/L), ultimately requiring combination prednisolone and rituximab therapy after steroid-dependent relapse.
- Initial presentation included jaundice, 7 kg weight loss over six weeks, and imaging showing pancreatic head mass with bile duct stenosis initially suspicious for malignancy, but endosonographic biopsy revealed no cancer and instead showed up to 15 IgG4-positive plasma cells per high power field with storiform fibrosis and obliterative phlebitis
- The speaker reported initial treatment with prednisolone 40 mg daily for four weeks produced clinical response and was tapered to 5 mg daily maintenance, but the patient relapsed with cholestasis and rising IgG4 levels in September representing steroid-dependent disease
- Re-induction therapy included prednisolone 40 mg daily plus rituximab 1 gram administered at two-week intervals, with the speaker noting rituximab is off-label and required insurance approval
- The speaker stated the patient experienced multiple infectious complications including cholangitis episodes requiring IV antibiotics and repeat ERCP with stent placement, causing treatment delays
- ERCP at end of February after rituximab therapy showed significant improvement of the biliary tree, with bile stones managed using ursodeoxycholic acid (UDCA) initiated early to prevent recurrence
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