Secondary Sclerosing Cholangiopathies

This review synthesizes recent evidence of secondary sclerosing cholangiopathies, specifically IgG4-sclerosing cholangiopathy, post-transplant cholangiopathies, COVID-19-induced cholangiopathy, and sclerosing cholangiopathies due to critical illness.

The clinical diagnostic criteria and practice guidelines have been updated for IgG4-sclerosing cholangiopathy. Cholangiopathy associated with livers donated after circulatory death has been further characterized, though incidence is expected to decline significantly as the use of normothermic perfusion technologies expands. COVID-19 sclerosing cholangiopathy, a likely novel entity similar in pathogenesity to sclerosing cholangiopathy of critical illness, has been identified during the SARS-CoV-2 pandemic.

The evaluation of progressive cholestasis requires consideration of rarer forms of secondary cholangiopathies based on clinical context.