Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
, 13 (3), 300-307

IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis


IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis

Atsushi Tanaka. Gut Liver.


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


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


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).

Similar articles

See all similar articles

Cited by 1 PubMed Central articles


    1. Lazaridis KN, LaRusso NF. Primary sclerosing cholangitis. N Engl J Med. 2016;375:1161–1170. doi: 10.1056/NEJMra1506330. - DOI - PMC - PubMed
    1. Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. Primary sclerosing cholangitis: a comprehensive review. J Hepatol. 2017;67:1298–1323. doi: 10.1016/j.jhep.2017.07.022. - DOI - PubMed
    1. Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T. Diagnosis of IgG4-related sclerosing cholangitis. World J Gastroenterol. 2013;19:7661–7670. doi: 10.3748/wjg.v19.i43.7661. - DOI - PMC - PubMed
    1. Tanaka A, Tazuma S, Nakazawa T, et al. No negative impact of serum IgG4 levels on clinical outcome in 435 patients with primary sclerosing cholangitis from Japan. J Hepatobiliary Pancreat Sci. 2017;24:217–225. doi: 10.1002/jhbp.432. - DOI - PubMed
    1. Tanaka A, Tazuma S, Okazaki K, et al. Clinical features, response to treatment, and outcomes of IgG4-related sclerosing cholangitis. Clin Gastroenterol Hepatol. 2017;15:920–926.e3. doi: 10.1016/j.cgh.2016.12.038. - DOI - PubMed

MeSH terms

LinkOut - more resources