摘要
Autoimmune pancreatitis(AIP)is a rare,heterogeneous,fibroinflammatory disorder of the pancreas.It has gained increasing recognition due to a presentation that can mimic difficult-to-treat disorders such as pancreatic cancer,cholangiocarcinoma and primary sclerosing cholangitis.In contrast,autoimmune pancreatitis is a benign disease that is very responsive to therapy with corticosteroids.There are two types of AIP.Type 1 disease is the most common worldwide and is associated with extrapancreatic manifestations and elevated levels of IgG4-positive cells.Type 2 AIP is characterized by a paucity of IgG4-positive cells and is more difficult to diagnose.This review provides an update on the diagnosis,pathophysiology and treatment of AIP,with special emphasis on the two subtypes.
