Table 4.
Type 1 AIP | ||
Criterion |
Level 1 |
Level 2 |
Parenchymal imaging |
Typical: Diffuse enlargement with delayed enhancement |
Indeterminate: Focal enlargement with delayed enhancement |
Ductal imaging (ERP) |
Long or multiple strictures (> 1/3 duct length) without upstream dilatation |
Focal narrowing without upstream dilatation (< 5 mm) |
Serology |
IgG4 > 2x upper limit |
IgG4 1-2x upper limit |
Other organ involvement |
Extrapancreatic organ histology. Any 3 of : 1 Lymphoplasmacytic infiltration with fibrosis and without granulocytic infiltration 2 Storiform fibrosis 3 Obliterative phlebitis 4 > 10 cells/HPF IgG4-positive cells Typical radiology. Any one of: 1 Segmental/multiple proximal or distal biliary stricture 2 Retroperitoneal fibrosis |
Extrapancreatic organ histology including bile duct biopsies. Both of: 1 Marked lymphoplasmacytic infiltration without granulocytic infiltration 2 10 cells/HPF IgG4-positive cells Physical or radiological evidence of at least one of: 1 Enlarged salivary/lachrymal glands 2 Renal involvement |
Histology of pancreas |
LPSP and 3 of: 1 Periductal lymphoplasmacytic infiltrate without granulocytic infiltration 2 Obliterative phlebitis 3 Storiform fibrosis 4 > 10 cells/HPF IgG4-positive cells |
LPSP and 2 of: 1 Periductal lymphoplasmacytic infiltrate without granulocytic infil tration 2 Obliterative phlebitis 3 Storiform fibrosis 4 > 10 cells/HPF IgG4-positive cells |
Response to steroid (Rt) |
Rapid (< 2 wk) radiological demonstration of marked improvement in pancreatic/extrapancreatic manifestations |
|
Type 2 AIP | ||
Parenchymal imaging |
Typical: Diffuse enlargement with delayed enhancement |
Indeterminate: Focal enlargement with delayed enhancement |
Ductal Imaging (ERCP) |
Long (> 1/3 duct length) or multiple strictures without upstream dilatation |
Focal narrowing without marked upstream dilatation (< 5 mm) |
Other organ involvement |
Clinically diagnosed inflammatory bowel disease |
|
Histology of pancreas |
IDCP. Both of: 1 Granulocytic infiltration of duct wall with or without acinar inflammation 2 0-10 cells/HPF IgG4-positive cells |
Both of : 1 Granulocytic and lymphoplasmacytic acinar infiltrate 2 0-10 cells/HPF IgG4-positive cells |
Response to steroid (Rt) | Rapid (< 2 wk) radiological demonstration of marked improvement in manifestations |
LPSP: Lymphoplasmacytic sclerosing pancreatitis; IDCP: Idiopathic duct-centric pancreatitis; AIP: Autoimmune pancreatitis; IgG4: immunoglobulin G4; ERP: Endoscopic retrograde pancreatography; Rt: Response to steroid therapy; HPF: High powered field.