Review Article

IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease

Table 2

Revised criteria of the Japan Biliary Association [71].

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 findingsElevated 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 AIPTypes 1, 2Ia/b + IIa/b + III/VI
Types 3, 4Ia + IIa + IVb + III/VI

Definite diagnosis isolated IgG4-SCTypes 1, 2, 3, 4Ia + IIa + III + IVa/VI

Probable diagnosis IgG4-SC associated with AIPTypes 1, 2
Types 3, 4
Ia/b + IIa/b
Ia + IIa + IVb
Ia/b + IIb + VI

Probable diagnosis isolated IgG4-SCTypes 1, 2, 3, 4Ia + IIa + Iva
Ia + IIa + III + IVb
Ib + IIa + III + VI

Possible diagnosis IgG4-SC associated with AIPTypes 3, 4Ia/b + IIa Ib + IIb + III

Possible diagnosisTypes 1, 2, 3, 4Ia + IIa + III/Vb/VI
Isolated IgG4-SCIb + IIb + III + VI