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Table 2 Mayo Clinic diagnostic groups with HISORt criteria (3,5)

From: Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

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.

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.