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Current Hepatology Reports

Erschienen in:

20.07.2020 | Autoimmune, Cholestatic, and Biliary Diseases (S Gordon and CL Bowlus, Section Editors)

IgG4-Related Sclerosing Cholangitis

verfasst von: Akash Singh, Virendra Singh

Erschienen in: Current Hepatology Reports | Ausgabe 3/2020

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Abstract

Purpose of Review

IgG4-related sclerosing cholangitis (ISC) is a frequent occurrence. However, its diagnosis is difficult. This review summarizes the clinical features, pathogenesis, differential diagnosis, and management of ISC.

Recent Findings

A precise diagnosis of ISC is important. Diagnosis is based on the Japanese criteria which has recently been provided. The characteristic features of plasma cell infiltration and raised IgG4 levels along with classical histopathological and imaging findings help in diagnosis. Steroid therapy is very effective in ISC. Immunomodulatory drugs have also shown promising results.

Summary

Diagnostic approach of ISC mainly involves imaging modalities. Japanese diagnostic criteria is important in its diagnosis. It is also extremely important to differentiate IgG4 sclerosing cholangitis from various other cholangiopathies. Steroid therapy stays the treatment of choice. The role of other immunomodulators needs to be researched and reciprocated in clinical setting before it replaces steroid therapy.

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Titel: IgG4-Related Sclerosing Cholangitis
verfasst von: Akash Singh
Virendra Singh
Publikationsdatum: 20.07.2020
Verlag: Springer US
Erschienen in: Current Hepatology Reports / Ausgabe 3/2020
Elektronische ISSN: 2195-9595
DOI: https://doi.org/10.1007/s11901-020-00535-9

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