Like autoimmune pancreatitis (AIP), IgG4-related sclerosing cholangitis (IgG4-SC) responds well to steroids, and serum IgG4 falls simultaneously during treatment. Steroid treatment is divided as induction phase, tapering phase, and maintenance phase. Although the starting dose of steroids is not established yet worldwide, it would be reasonable to use the same drug regimen as for AIP, starting at 0.6 mg/kg/day (or 30–60 mg/day) and titrating down the dose as the laboratory results including serum IgG4 improve. A response is expected within 2–4 weeks of steroid treatment. On the other hand, opinion for maintenance treatment is different between Asian and Western countries. The dose is usually tapered to a maintenance dose (2.5–5 mg/day) over a period of 2–3 months, based on changes in clinical manifestations, biochemical blood tests, and imaging findings. Cessation of steroid therapy should be based on the disease activity in each case. The initial response rate to steroid of IgG4-SC was up to 90–97%.
KeywordsIgG4-related sclerosing cholangitis Autoimmune pancreatitis IgG4-related disease Treatment Steroid
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