Table 8.

Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012 (from [66], with permission)

Diagnostic items
(1) Biliary tract imaging reveals diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct associated with the thickening of bile duct wall
(2) Hematological examination shows elevated serum IgG4 concentrations (≥135 mg/dl)
(3) Coexistence of autoimmune pancreatitis, IgG4-related dacryoadenitis/sialadenitis, or IgG4-related retroperitoneal fibrosis
(4) Histopathological examination shows:
a. Marked lymphocytic and plasmacyte infiltration and fibrosis
b. Infiltration of IgG4-positive plasma cells:[10 IgG4-positive plasma cells/HPF
c. Storiform fibrosis
d. Obliterative phlebitis
<Option> effectiveness of steroid therapy
A specialized facility, in which detailed examinations such as endoscopic biliary biopsy and endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) can be administered, may include in its diagnosis the effectiveness of steroid therapy, once pancreatic or biliary cancers have been ruled out.
Diagnosis
Definite diagnosis
(1) + (3)
(1) + (2) + (4) a, b
(4) a, b, c
(4) a, b, d
Probable diagnosis
(1) + (2) + option
Possible diagnosis
(1) + (2)

It is necessary to exclude PSC, malignant diseases such as pancreatic or biliary cancers, and secondary sclerosing cholangitis caused by the diseases with obvious pathogenesis. When it is difficult to differentiate from malignant conditions, a patient must not be treated with facile steroid therapy, but should be referred to a specialized medical facility