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Autoimmune pancreatitis is an increasingly recognized inflammatory type of subacute pancreatitis; two subtypes of autoimmune pancreatitis have been identified so far: the “lymphoplasmacytic” type 1 variant and the “neutrophilic” type 2 variant. Type 1 autoimmune pancreatitis represents the most common manifestation of IgG4‐related disease, a fibro‐inflammatory disorder characterized by elevated IgG4 levels in the serum and affected tissues. Type 2 autoimmune pancreatitis is a pancreas‐specific disorder that frequently occurs in the context of inflammatory bowel diseases. Due to the complexity of both diseases, a comprehensive work up with imaging, laboratory, and histological studies is required to achieve a diagnosis and rule out malignancies. Glucocorticoids represent the cornerstone of the treatment, often supported by other immunosuppressive drugs in case of steroid intolerance or aggressive disease. Maintenance treatment is often employed in type 1 autoimmune pancreatitis because of the higher relapse rate compared with type 2 autoimmune pancreatitis. In this review, we summarize the key concept of autoimmune pancreatitis, delve into the differential diagnosis between the two subtypes, and cover the recent relevant research findings and pressing unmet needs.
Autoimmune pancreatitis is an increasingly recognized inflammatory type of subacute pancreatitis; two subtypes of autoimmune pancreatitis have been identified so far: the “lymphoplasmacytic” type 1 variant and the “neutrophilic” type 2 variant. Type 1 autoimmune pancreatitis represents the most common manifestation of IgG4‐related disease, a fibro‐inflammatory disorder characterized by elevated IgG4 levels in the serum and affected tissues. Type 2 autoimmune pancreatitis is a pancreas‐specific disorder that frequently occurs in the context of inflammatory bowel diseases. Due to the complexity of both diseases, a comprehensive work up with imaging, laboratory, and histological studies is required to achieve a diagnosis and rule out malignancies. Glucocorticoids represent the cornerstone of the treatment, often supported by other immunosuppressive drugs in case of steroid intolerance or aggressive disease. Maintenance treatment is often employed in type 1 autoimmune pancreatitis because of the higher relapse rate compared with type 2 autoimmune pancreatitis. In this review, we summarize the key concept of autoimmune pancreatitis, delve into the differential diagnosis between the two subtypes, and cover the recent relevant research findings and pressing unmet needs.
Objectives: Autoimmune pancreatitis (AIP) type 1, paraduodenal (groove) pancreatitis, and follicular pancreatitis are rare clinical entities whose diagnosis may be challenging, given the potential imaging overlap with pancreatic cancer. Methods: We performed a retrospective analysis of the medical chart of a patient with multiple pancreas pathologies. Results: We present a case with multiple pancreas pathologies, including a poorly differentiated ductal adenocarcinoma of pancreatobiliary type, an intraductal papillary mucinous lesion (pre-existing lesion of IPMN type), and an inflammatory process with complex features, in which paraduodenal (groove) pancreatitis, follicular pancreatitis, and IgG4-related pancreatitis (AIP type 1) were also present. Conclusions: The diagnosis of AIP and paraduodenal pancreatitis is not always straightforward, and in some cases, it is not easy to differentiate them from pancreatic cancer. Surgery should be considered in patients when a suspicion of malignant/premalignant lesions cannot be excluded after a complete diagnostic work-up.
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