Although autoimmune pancreatitis is a relatively uncommon pancreatic disorder, it is an important consideration in the differential diagnosis of jaundice and pancreatic masses to avoid unnecessary surgical resection. The clinical diagnosis of autoimmune pancreatitis involves considering multiple pieces of information including the pancreatic imaging, serum IgG4 level, presence of characteristic other involvement, and histologic findings. Unlike other pancreatic disorders, this condition rapidly responds to treatment with steroids. In the more common disease subtype (type 1 autoimmune pancreatitis) disease relapses can occur necessitating the use of maintenance treatment with low-dose steroids, steroid-sparing immunomodulators, or rituximab. This chapter reviews the diagnosis and treatment of the autoimmune pancreatitis subtypes.
Keywords: Azathioprine; Idiopathic ductcentric chronic pancreatitis; IgG4-related disease; Lymphoplasmacytic sclerosing pancreatitis; Prednisone; Rituximab.