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| HISORt criteria | Japan Biliary Association criteria |
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(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 |
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(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 |
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(3) Serology | Raised serum IgG4 levels (>1.35 g/L) | Elevated serum IgG4 concentrations (≥135 mg/dL) |
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(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 |
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(5) Response to steroids | Normalization of liver enzymes and at least partial stricture resolution after steroid treatment | Effectiveness of steroid therapy |
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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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