IgG4-related sclerosing cholangitis (IgG4-SC) is a biliary manifestation of a systemic condition known as IgG4-related disease (IgG4-RD). IgG4-RD may involve various organs with type 1 autoimmune pancreatitis (AIP) being the leading manifestation. Older men are more affected by IgG4-SC than women (ratio of 4:1), and the most common clinical manifestation is obstructive jaundice. The pathogenic mechanism of IgG4-SC remains unclear. Steroid therapy can lead to clinical and radiological improvement when provided in the inflammatory phase of this disease. However, long-term outcome data in patients with IgG4-SC are lacking. In this chapter, we focus on the prognosis of IgG4-SC, including the natural course, treatment response, risk of malignancy, and long-term outcome, based on current evidence.
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