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IgG4-Related Sclerosing Cholangitis

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Abstract

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of a multi-organ chronic fibroinflammatory condition, IgG4-related disease. Clinically it may mimic other biliary disease, including primary sclerosing cholangitis and cholangiocarcinoma. IgG4-SC may lead to progressive fibrosclerotic disease and cirrhosis. There is no specific test to make the diagnosis, which is based on clinical, laboratory, radiological and pathological features. Steroids and other immunosuppressive therapy may lead to clinical and radiological improvement when given in the early phase of disease, but the clear evidence base for treatment regimens is limited. Over the last decade, progress has been made in understanding the immunopathogenesis of IgG4-SC, including the role of HLA class II susceptibility molecules, circulating memory B cells and plasmablasts, T helper 2 and regulatory cells, chemokine-mediated trafficking and the innate immune system.

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Correspondence to George Webster MBBS, MD, FRCP .

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Culver, E.L., Webster, G. (2017). IgG4-Related Sclerosing Cholangitis. In: Hirschfield, G., Adams, D., Liaskou, E. (eds) Biliary Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-50168-0_12

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