Group A: | Diagnostic pancreatic histology (presence of 1 or both of the following): Resection specimen or core biopsy showing the full spectrum of changes of LPSP, and/or ≥ 10 IgG4 positive cells/hpf; IgG4 on immunostain of pancreatic lymphoplasmacytic infiltrate. |
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Group B: | Typical imaging + serology (presence of all of the following criteria): CT or MRI showing diffusely enlarged pancreas with delayed and "rim" enhancement; pancreatogram showing diffusely irregular pancreatic duct; elevated serum IgG4 levels. |
Group C: | Response to steroids (presence of all of the following criteria): Unexplained pancreatic disease after negative work-up for known etiologies including cancer; elevated serum IgG4 levels and/or other organ involvement confirmed by presence of abundant IgG4-positive cells; resolution/marked improved in pancreatic and/or extrapancreatic manifestations with steroid therapy. |