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Purpose of Review: 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. Recent Findings: 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. Summary: The evaluation of progressive cholestasis requires consideration of rarer forms of secondary cholangiopathies based on clinical context. [ABSTRACT FROM AUTHOR]

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection. [ABSTRACT FROM AUTHOR]