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Review
, 13 (3), 300-307

IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis

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Review

IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis

Atsushi Tanaka. Gut Liver.

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.

Keywords: Cholangiography; Cholangitis; Corticosteroid; IgG4.

Conflict of interest statement

CONFLICTS OF INTEREST

No potential conflict of interest relevant to this article was reported.

Figures

Fig. 1
Fig. 1
Age distribution at presentation. (A) IgG4-related sclerosing cholangitis and (B) primary sclerosing cholangitis.
Fig. 2
Fig. 2
Distribution of serum IgG4 levels at presentation. (A) IgG4-related sclerosing cholangitis and (B) primary sclerosing cholangitis.
Fig. 3
Fig. 3
Typical cholangiographic features of primary sclerosing cholangitis. (A) Beaded appearance (arrows), (B) pruned tree appearance, and (C) band-like stricture (arrows).

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