Autoimmune pancreatitis is a distinct form of chronic pancreatitis that is characterized by a dramatic response to steroid treatment. In addition to resolution of the pancreatic manifestations, disease activity in other affected organs is also steroid responsive. The primary goal of treatment is reduction in acute inflammation and hopefully to prevent progression to irreversible fibrosis. Initial treatment also often requires management of diabetes mellitus and biliary obstruction. Though initial response to steroids is invariable, relapses are common. Nearly 50 % of patients relapse after initial therapy if no maintenance treatment is given, particularly those with involvement of the proximal biliary tract. Disease relapses generally occur in the pancreas or biliary tree; however, they can also develop in other previously unaffected organs. The remission rates using steroid treatment for disease relapses remain very high. There are several options for preventing relapses including long-term low-dose steroids, steroid-sparing immunomodulators, and rituximab; however, data supporting these strategies is just emerging.
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