|
| I. | Narrowing of the intrahepatic and/or extrahepatic bile duct | (a) ERC |
| (b) MRCP |
|
| II. | Thickening of the bile duct wall | (a) EUS/IDUS |
| (b) CT/MRI/US |
|
| III. | Serological findings | Elevated serum IgG4 concentrations (≥135 mg/dL) |
|
| 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 |
| |
| (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 |
|
| V. | Other organ involvement (OOI) | (a) Type 1 autoimmune pancreatitis |
| (b) IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, IgG4-related kidney lesion |
|
| VI. | Effectiveness of steroid therapy | |
|
| Definite diagnosis IgG4-SC associated with AIP | Types 1, 2 | Ia/b + IIa/b + III/VI |
| Types 3, 4 | Ia + IIa + IVb + III/VI |
|
| Definite diagnosis isolated IgG4-SC | Types 1, 2, 3, 4 | Ia + IIa + III + IVa/VI |
|
| Probable diagnosis IgG4-SC associated with AIP | Types 1, 2
Types 3, 4 | Ia/b + IIa/b |
| Ia + IIa + IVb |
| Ia/b + IIb + VI |
|
| Probable diagnosis isolated IgG4-SC | Types 1, 2, 3, 4 | Ia + IIa + Iva |
| Ia + IIa + III + IVb |
| Ib + IIa + III + VI |
|
| Possible diagnosis IgG4-SC associated with AIP | Types 3, 4 | Ia/b + IIa Ib + IIb + III |
|
| Possible diagnosis | Types 1, 2, 3, 4 | Ia + IIa + III/Vb/VI |
| Isolated IgG4-SC | Ib + IIb + III + VI |
|
