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I. | Narrowing of the intrahepatic and/or extrahepatic bile duct | (a) ERC |
(b) MRCP |
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II. | Thickening of the bile duct wall | (a) EUS/IDUS |
(b) CT/MRI/US |
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III. | Serological findings | Elevated serum IgG4 concentrations (≥135 mg/dL) |
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IV. | Pathological findings among (i)–(v) listed below | (a) (i), (ii), and (v) are observed |
(b) (v) is observed |
(c) All of (i), (ii), and (v) and either or both of (iii) or (iv) are observed |
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(i) Marked lymphoplasmacytic infiltration and fibrosis |
(ii) More than 10 IgG4-positive plasma cells per high-power microscopic field |
(iii) Storiform fibrosis |
(iv) Obliterative phlebitis |
(v) No neoplastic cells identified |
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V. | Other organ involvement (OOI) | (a) Type 1 autoimmune pancreatitis |
(b) IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, IgG4-related kidney lesion |
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VI. | Effectiveness of steroid therapy | |
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Definite diagnosis IgG4-SC associated with AIP | Types 1, 2 | Ia/b + IIa/b + III/VI |
Types 3, 4 | Ia + IIa + IVb + III/VI |
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Definite diagnosis isolated IgG4-SC | Types 1, 2, 3, 4 | Ia + IIa + III + IVa/VI |
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Probable diagnosis IgG4-SC associated with AIP | Types 1, 2 Types 3, 4 | Ia/b + IIa/b |
Ia + IIa + IVb |
Ia/b + IIb + VI |
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Probable diagnosis isolated IgG4-SC | Types 1, 2, 3, 4 | Ia + IIa + Iva |
Ia + IIa + III + IVb |
Ib + IIa + III + VI |
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Possible diagnosis IgG4-SC associated with AIP | Types 3, 4 | Ia/b + IIa Ib + IIb + III |
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Possible diagnosis | Types 1, 2, 3, 4 | Ia + IIa + III/Vb/VI |
Isolated IgG4-SC | Ib + IIb + III + VI |
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