Abstract:Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis which belongs to the spectrum of IgG4 related diseases.AIP is characterized by lymphoplasmacytic infiltration,storiform fibrosis,obliterative phlebitis,and increasing of IgG4+ plasma cells.Patients with AIP presented clinically with recurrent pancreatitis and obstructive jaundice,usually with symptoms involving bile duct and salivary glands and so on.Serum IgG4 level is often elevated.The main imaging appearance of AIP is a “sausage-shaped pancreas” and multiple stricture of the main pancreatic duct.AIP responds dramatically to glucocorticosteroid but relapses easily.In relapsed cases,re-administration of steroid or in combination with immunosuppressant is effective.