Auto-immune pancreatitis is a chronic immune-mediated fibroinflammatory disease primarily involving pancreas responding to steroid therapy.
Mayo Clinic HISORt Criteria for IgG4 Autoimmune Pancreatitis
Histology:
(1) Periductal lymphoplasmacytic infiltrate with obliterative phlebitis and storiform fibrosis (LPSP) OR
(2) Lymphoplasmacytic infiltrate with storiform fibrosis showing abundant (210 cells/HPF) IgG4-positive cells
Imaging:
Typical: diffusely enlarged gland with delayed (rim) enhancement; diffusely irregular, attenuated main pancreatic duct
Others: Focal pancreatic mass/enlargement; focal pancreatic duct stricture; pancreatic atrophy; pancreatic calcification; or pancreatitis
Serology: Elevated serum IgG4 level (>135 mg/dL)
Other Organ Involvement.
Response to steroids.
Type 1 (lymphoplasmacytic sclerosing pancreatitis) and type 2 (idiopathic duct-centric chronic pancreatitis).
IgG4 disease is a multi-system disorder. Following disorders have been described (Common disorders are highlighted):
IgG4 pancreatitis responds well to corticosteroids.
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