Abstract
Autoimmune pancreatitis (AIP) can be defined as a rare chronic inflammation of the pancreas due to an autoimmune mechanism. Corticosteroid treatment and the long-term prognosis (outcome) of AIP are still controversial. In Japan, the initial corticosteroid dosage has been 30 to 40mg per day and tapered during one to two months, based on changes in clinical symptoms, laboratory data and abdominal imaging of the pancreas and bile duct. Recently, cases of malignancies including pancreatic cancer have been reported during the course of AIP. However, the carcinogenesis of AIP is not understood. Further studies are necessary to clarify the initial corticosteroid dosage (30mg or 40mg) and the period of maintenance treatment as well as the long-term outcome of AIP. Furthermore, the natural history of AIP needs to be more clearly elucidated.