Gut Liver.  2019 May;13(3):300-307. 10.5009/gnl18085.

IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis

• Tanaka A

• KMID: 2448369
• DOI: http://doi.org/10.5009/gnl18085

Abstract

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. Cholangiography, endoscopic or magnetic, is inevitably required for making a diagnosis. Although the presentation of IgG4-SC and PSC are similar, the comorbidities, treatment response, and outcomes differ significantly, and therefore, it is strongly advisable to be familiar with these two diseases to make a correct diagnosis. Differentiation of cholangiocarcinoma from IgG4-SC and PSC is also extremely important. In this review, the clinical characteristics, comorbidities, treatment and outcomes of IgG4-SC and PSC will be outlined based on experience mainly from Japan.

Keyword

IgG4; Cholangiography; Cholangitis; Corticosteroid

MeSH Terms

Bile Ducts
Cholangiocarcinoma
Cholangiography
Cholangitis
Cholangitis, Sclerosing*
Cholestasis
Comorbidity
Constriction, Pathologic
Diagnosis
Fibrosis
Hematologic Tests
Humans
Immunoglobulin G
Inflammation
Japan
Jaundice
Pruritus
Immunoglobulin G