What is the recommended medical management of primary sclerosing cholangitis, and is there a role for corticosteroids?
Medically reviewed by Louis-Antoine Mullie, MD.
Answer
Medical management of primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, leading to progressive liver dysfunction and an increased risk of malignancies . The management of PSC is primarily supportive, as no single drug has been proven to improve transplant-free survivalRole of corticosteroids
- General use: Corticosteroids are not recommended for routine use in the management of PSC. The EASL 2022 guidelines advise against their use in patients with classic PSC . This is supported by the BSG 2019 guidelines, which also recommend against the use of corticosteroids in classic PSC
- Specific conditions: Corticosteroids may be considered in certain conditions. For instance, the EASL 2022 guidelines suggest their use in patients with PSC who have biochemically and histologically suggestive features of autoimmune hepatitis (AIH) . Additionally, the BSG 2019 guidelines suggest corticosteroids in patients with additional features of AIH or IgG4-related sclerosing cholangitis (IgG4-SC)
- IgG4-related sclerosing cholangitis: In the case of IgG4-SC, corticosteroids are recommended as first-line therapy. The JSHBPS 2019 guidelines suggest initiating corticosteroids in almost all patients with IgG4-SC, especially those with obstructive jaundice, acute cholangitis, or symptomatic extrabiliary IgG4-related disease
Management of pruritus
Pruritus is a common symptom in PSC, and several guidelines provide recommendations for its management:
- Initial therapy: Bile acid sequestrants, such as cholestyramine, are recommended as initial therapy for pruritus in PSC by the AASLD 2022 guidelines and the ACG 2015 guidelines
- Second-line therapy: If cholestyramine is ineffective or poorly tolerated, second-line treatments such as rifampin and naltrexone are recommended by the ACG 2015 guidelines and the BSG 2019 guidelines
- Refractory pruritus: For refractory pruritus, the AASLD 2022 guidelines suggest alternatives such as sertraline, naltrexone, and rifampin
In conclusion, the management of PSC is primarily supportive, with corticosteroids not recommended for routine use. However, they may be considered in specific conditions such as AIH or IgG4-SC. Pruritus, a common symptom in PSC, can be managed with bile acid sequestrants, with second-line treatments available if necessary.