|
| (1) (P) Pancreatic enlargement | |
| Level 1: typical imaging, diffuse | |
| Level 2: indeterminate imaging, segmental/focal | |
| (2) (D) Narrowing of the main pancreatic duct on ERP | |
| Level 1: >1/3 length or multiple stricture | |
| Level 2: segmental/focal | |
| (3) (S) Serum IgG4 level | |
| Level 1: >270 mg/dL | |
| Level 2: 135–270 mg/dL | |
| (4) (OOI) Other organ involvement | |
| Level 1: histology (≥3 criteria), or sclerosing cholangitis, or retroperitoneal fibrosis | |
| Level 2: histology (2 criteria), or salivary/lacrimal gland swelling, or renal involvement | |
| (5) (H) Histology of the pancreas (LPSP) | |
| Level 1: ≥3 of 4 criterions (periductal lymphoplasmacytic infiltrate without granulocytic infiltration, obliterative phlebitis, storiform fibrosis, and abundant IgG4-positive cells) | |
| Level 2: 2 criteria | |
| (6) (Rt) Response to steroid | |
|
| Definite type 1 diagnosis | |
| Level 1 histology | |
| Typical imaging: any non-D level 1/level 2 | |
| Indeterminate imaging: two or more from level 1 + level 2 D | |
| level 1 S/OOI + Rt or level 1 D + level 2 S/OOI/H + Rt | |
| Probable type 1 diagnosis | |
| Indeterminate imaging: level 2 S/OOI/H + Rt | |
| AIP-NOS | |
| D1/2 + Rt | |
|
