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Review of primary sclerosing cholangitis with increased IgG4 levels by Charis D Manganis; Roger W Chapman; Emma L Culver is a Medicine article available to read on EtoBox.

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Primary sclerosing cholangitis (PSC) is a chronic progressive liver disease. Sub-types of PSC have been described, most recently PSC with elevated serum and/or tissue IgG4 subclass. We aim to summarise the clinical phenotype, disease associations, differential diagnosis, response to therapy and pathogenic mechanisms underlying PSC-high IgG4 subtype. We reviewed PubMed, MEDLINE and Embase with the search terms “primary sclerosing cholangitis”, “IgG4”, and “IgG4-related sclerosing cholangitis (IgG4-SC)”. Elevated serum IgG4 are found in up-to one-quarter, and abundant IgG4-plasma cell infiltrates in the liver and bile ducts are found in up-to one-fifth of PSC patients. This group have a distinct clinical phenotype, with some studies reporting a more aggressive course of liver and associated inflammatory bowel disease, compared to PSC-normal IgG4 and the disease mimic IgG4-SC. Distinguishing PSC-high IgG4 from IgG4-SC remains challenging, requiring careful assessment of clinical features, organ involvement and tissue morphology. Calculation of serum IgG~4~:IgG~1~ ratios and use of a novel IgG~4~:IgG RNA ratio have been reported to have excellent specificity to distinguish IgG4-SC and

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Author

Charis D Manganis; Roger W Chapman; Emma L Culver

Publisher

Baishideng Publishing Group Inc.

Published

2020

Language

EN

Field

Medicine (Health Sciences)