IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis

Overview

Journal Gut Liver

Specialty Gastroenterology

Date 2018 Sep 12

PMID 30205418

Citations 22

Authors

Atsushi Tanaka

Affiliations

Soon will be listed here.

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.

Citing Articles

Beyond cholangiocarcinoma: imaging features of mimicking pathologies in the biliary tract.

Khasawneh H, OBrien C, Czeyda-Pommersheim F, Qayyum A, Miller F, Arif Tiwari H Abdom Radiol (NY). 2024; .

PMID: 39710762 DOI: 10.1007/s00261-024-04749-z.

Renal Manifestations of IgG4-Related Disease: A Concise Review.

Towheed S, Zanjir W, Ren K, Garland J, Clements-Baker M Int J Nephrol. 2024; 2024:4421589.

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Recombinant adeno-associated virus 8-mediated inhibition of microRNA let-7a ameliorates sclerosing cholangitis in a clinically relevant mouse model.

Hua H, Zhao Q, Kalagbor M, Yu G, Liu M, Bian Z World J Gastroenterol. 2024; 30(5):471-484.

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An atypical case of isolated immunoglobulin G4-related sclerosing cholangitis with a cholangiogram resembling primary sclerosing cholangitis.

Takada Y, Ishikawa T, Yamao K, Mizutani Y, Iida T, Uetsuki K Clin J Gastroenterol. 2024; 17(2):338-344.

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Wu S, Wang H Front Immunol. 2023; 14:1278332.

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