Abstract: Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis, which is clinically divided into two types (type Ⅰ and type Ⅱ) according to histology. Type Ⅰ is the main disease in China, which is a part of the IgG4-related disease characterized clinically by obstructive jaundice or a pancreatic mass, histologically by numerous lymphoplasmacytic infiltrate and chronic fibrosis. Although the International Association of Pancreatology (IAP) has developed diagnostic criteria for its imaging, serology, histology and hormone therapy responses, the clinical features of autoimmune pancreatitis are occult and varied, the diagnosis of autoimmune pancreatitis remains challenging, particularly in patients with pancreatic masses, which need to be alert to the risk of the pancreatic cancer. This review is to summarize the literature from the perspective of image and histology, focusing on the diagnosis and risk of misdiagnosis of this complex disease, in order to improve the diagnosis of autoimmune pancreatitis.

Key words: autoimmune diseases, pancreatitis, review, immunoglobulinG4 related disease, autoimmune pancreatitis