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| | HISORt criteria | Japan Biliary Association criteria |
|
| (1) Other organ involvement | Extrabiliary manifestations consistent with IgG4-RD, such as pancreas (focal pancreatic mass/enlargement without pancreatic duct dilatation, multiple pancreatic masses, focal pancreatic duct stricture without upstream dilatation, pancreatic atrophy);
Retroperitoneal fibrosis;
Kidney (single or multiple parenchymal low attenuation lesions: Round, wedge-shaped, or diffuse patchy);
Salivary or lacrimal gland (enlargement) | Coexistence of autoimmune pancreatitis, or IgG4-related dacryoadenitis/sialadenitis, or IgG4-related retroperitoneal fibrosis |
|
| (2) Histology | Lymphoplasmacytic infiltrate with >10 IgG4+ cells per high-power field within and around bile ducts;
Obliterative phlebitis;
Storiform fibrosis | (a) Marked lymphocytic and plasmacyte infiltration and fibrosis |
| (b) Infiltration of IgG4-positive plasma cells >10 IgG4-positive plasma cells/HPF |
| (c) Storiform fibrosis, obliterative phlebitis |
|
| (3) Serology | Raised serum IgG4 levels (>1.35 g/L) | Elevated serum IgG4 concentrations (≥135 mg/dL) |
|
| (4) Imaging | Strictures of the biliary tree including intrahepatic ducts, proximal extrahepatic ducts, intrapancreatic ducts; fleeting and migrating biliary strictures | Diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct associated with the thickening of bile duct wall |
|
| (5) Response to steroids | Normalization of liver enzymes and at least partial stricture resolution after steroid treatment | Effectiveness of steroid therapy |
|
| Definite IgG4-SC | 2 + 4, 3 + 4 | 1 + 4, 2a & b+3 + 4, 2a & b & c, 2a & b & d |
| Probable IgG4-SC | 2 of the following: 1, 3, partial 2, partial 4 | 3 + 4+5 |
| Possible IgG4-SC | N/A | 3 + 4 |
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