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What is the recommended medical management of primary sclerosing cholangitis, and is there a role for corticosteroids?

Medically reviewed by Hovhannes Karapetyan, MD.

Answer

Medical management of primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis
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Abstract

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Primary sclerosing cholangitis

PubMed
Highly cited
. The management of PSC is primarily supportive, as no single drug has been proven to improve transplant-free survival
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Role of corticosteroids

The use of corticosteroids in PSC is generally not recommended. The EASL 2022 guidelines advise against the routine use of corticosteroids in PSC patients . However, in patients with PSC with biochemically and histologically suggestive features of AIH, initiating corticosteroids under close monitoring can be considered . Additionally, in patients with additional features of AIH or IgG4-related sclerosing cholangitis (IgG4-SC), corticosteroids can be considered

IgG4-related sclerosing cholangitis

In the case of IgG4-SC, corticosteroids are recommended as first-line therapy. The EASL 2022 guidelines recommend initiating prednisone/prednisolone (0.5-0.6 mg/kg/day) for untreated active IgG4-related cholangitis, with treatment response evaluated after 2-4 weeks before tapering . The JSHBPS 2019 guidelines also recommend initiating corticosteroids in almost all patients with IgG4-SC, with dose reduction or discontinuation considered carefully after remission has been maintained for 3 years

References

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Lancet 2018

Abstract

Primary sclerosing cholangitis

PubMed
Highly cited