Clinical Diagnostic Criteria of IgG4-Related Sclerosing Cholangitis (IgG4-SC)

Sclerosing cholangitis (SC) is defined as a condition with progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis and currently includes three categories: primary sclerosing cholangitis (PSC), secondary cholangitis, and IgG4-related sclerosing cholangitis (IgG4-SC). SC categories share similar clinical features, such as cholestasis. Patients with SC present with cholestatic symptoms, including jaundice and pruritus, and blood tests reveal elevation of cholestatic enzymes.

Clinical Diagnostic Criteria of IgG4-Related Sclerosing Cholangitis

Diagnostic items

1. Biliary tract imaging reveals diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct, associated with thickening of the bile duct wall
2. Hematological examination shows elevated serum IgG4 concentration (≥135 mg/dL)
3. Coexistence of autoimmune pancreatitis, IgG4-related dacryoadenitis/sialadenitis, or IgG4-related retroperitoneal fibrosis
4. Histopathological examination shows:

1. 1. Marked lymphocytic and plasmacytic infiltration and fibrosis
   2. Infiltration of IgG4-positive plasma cells (>10 cells per high power field)
   3. Storiform fibrosis
   4. Obliterative phlebitis

Optional: effectiveness of steroid therapy

Diagnosis

• Definite diagnosis: (1)+(3), or (1)+(2)+(4) (a+b, or a+b+c, or a+b+d)
• Probable diagnosis: (1)+(2)+optional item
• Possible diagnosis: (1)+(2)

 

References:

1. Tanaka A. IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis. Gut Liver. 2019 May 15;13(3):300-307. [Medline]
2. Tanaka A. Immunoglobulin G4-related sclerosing cholangitis. J Dig Dis. 2019 Jul;20(7):357-362. [Medline]

 

Created: Jan 27, 2021.