Criterion | Level 1 | Level 2 |
P | Parenchymal imaging | Typical: - Diffuse enlargement with delayed enhancement (sometimes associated with rim-like enhancement)
| Indeterminate (including atypical*): - Segmental/focal enlargement with delayed enhancement
|
D | Ductal imaging (ERP) | Long (greater than one-third length of the main pancreatic duct) or multiple strictures without marked upstream dilatation | Segmental/focal narrowing without marked upstream dilatation (duct size, <5 mm) |
S | Serology | IgG4, >2× upper limit of normal value | IgG4, 1 to 2× upper limit of normal value |
OOI | Other organ involvement | a or b | a or b |
- Histology of extrapancreatic organs
Any three of the following: - Marked lymphoplasmacytic infiltration with fibrosis and without granulocytic infiltration
- Storiform fibrosis
- Obliterative phlebitis
- Abundant (>10 cells/HPF) IgG4-positive cells
- Typical radiologic evidence
At least one of the following: - Segmental/multiple proximal (hilar/intrahepatic) or proximal and distal bile duct stricture
- Retroperitoneal fibrosis
| - Histology of extrapancreatic organs including endoscopic biopsies of bile duct¶
Both of the following: - Marked lymphoplasmacytic infiltration without granulocytic infiltration
- Abundant (>10 cells/HPF) IgG4-positive cells
- Physical or radiologic evidence
At least one of the following: - Symmetrically enlarged salivary/lachrymal glands
- Radiologic evidence of renal involvement described in association with AIP
|
H | Histology of the pancreas | LPSP (core biopsy/resection) | LPSP (core biopsy) |
At least 3 of the following: - Periductal lymphoplasmacytic infiltrate without granulocytic infiltration
- Obliterative phlebitis
- Storiform fibrosis
- Abundant (>10 cells/HPF) IgG4-positive cells
| Any 2 of the following: - Periductal lymphoplasmacytic infiltrate without granulocytic infiltration
- Obliterative phlebitis
- Storiform fibrosis
- Abundant (>10 cells/HPF) IgG4-positive cells
|
Response to steroid (Rt)Δ | Diagnostic steroid trial |
Rapid (≤2 weeks) radiologically demonstrable resolution or marked improvement in pancreatic/extrapancreatic manifestations |