|
| Criterion | Level 1 | Level 2 |
|
| Parenchymal imaging | Typical: diffuse enlargement with delayed enhancement (sometimes associated with rim-like enhancement) | Indeterminate (including atypical): segmental/focal enlargement with delayed enhancement |
|
| Ductal imaging (ERP) | Long (>1/3 length of the main pancreatic duct) or multiple strictures without marked upstream dilatation | Segmental/focal narrowing without marked upstream dilatation (duct size, <5 mm) |
|
| Serology | IgG4, >2x_upper limit of normal value | IgG4, 1-2x_upper limit of normal value |
|
| | a or b | a or b |
| | (a) Histology of extrapancreatic organs | (a) Histology of extrapancreatic organs including endoscopic biopsy of bile duct |
| | Any three of the following: | Both of the following: |
| | (1) Marked lymphoplasmacytic infiltration with fibrosis and without granulocytic infiltration | (1) Marked lymphoplasmacytic infiltration with fibrosis without granulocytic infiltration |
| | (2) Storiform fibrosis granulocytic infiltration | (2) Abundant (>10 cells/HPF) IgG4-positive cells |
| Other organ involvement (OOI) | (3) Obliterative phlebitis | |
| | (4) Abundant (>10 cells/HPF) IgG4-positive cells | |
| | (b) Typical radiological evidence | (b) Physical or radiological evidence |
| | At least one of the following: | At least one of the following: |
| | (1) Segmental/multiple proximal (hilar/intrahepatic) or proximal and distal bile duct stricture | (1) Symmetrically enlarged salivary/lacrimal glands |
| | (2) Retroperitoneal fibrosis | (2) Radiological evidence of renal involvement described in association with AIP |
|
| | LPSP (core biopsy/resection) | LPSP (core biopsy) |
| | At least 3 of the following: | Any 2 of the following: |
|
Histology of the pancreas | (1) Periductal lymphoplasmacytic infiltrate without granulocytic infiltration | (1) Periductal lymphoplasmacytic infiltrate without granulocytic infiltration |
| (2) Obliterative phlebitis | (2) Obliterative phlebitis |
| | (3) Storiform fibrosis | (3) Storiform fibrosis |
| | (4) Abundant (>10 cells/HPF) IgG4-positive cells | (4) Abundant (>10 cells/HPF) IgG4-positive cells |
|
