|
| Diagnosis | Primary basic for diagnosis | Imaging Evidence | Collateral evidence |
|
| Definitive type 1 AIP | Histology | Typical/indeterminate | Histologically confirmed LPSP (level 1 H) |
| Imaging | Typical | Any non-D level 1/level 2 |
| Indeterminate | Two or more from level 1 (+level 2 D*) |
| Response to steroid | Indeterminate | Level 1 S/OOI + Rt or level 1 D + level 2 S/OOI/H + Rt |
|
| Probable type 1 AlP | | Indeterminate | Level 2 S/OOI/H + Rt |
|
|
*Level 2 D is counted as level 1 in this setting. |
|
| 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 (>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) |
|
| S | Serology | IgG4, >2× upper limit of normal value a or b | IgG4, 1-2× upper limit of normal value a or b |
| OOI | Other organ involvement | | |
| | (a) Histology of extrapancreatic organs: | (a) Histology of extrapancreatic organs including endoscopic biopsies 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 without granulocytic infiltration; |
| | (2) storiform fibrosis; | (2) abundant (>10 cells/HPF) IgG4-positive cells. |
| | (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/lachrymal glands; |
| | (2) retroperitoneal fibrosis; | (2) radiological 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: | any 2 of the following: |
| | (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. |
|
| | Diagnostic steroid trial |
| Response to steroid (Rt)* |
Rapid (≤2 wk) radiologically demonstrable resolution or marked improvement in pancreatic/extrapancreatic manifestations |
|
