Abstract
Autoimmune Pancreatitis (AIP) is an emerging clinical entity found in 4.6-6 percent of patients with chronic pancreatitis [1]. It was first reported as an idiopathic chronic pancreatitis associated with hypergammaglobulinemia by Sarles et al. [2], with the term AIP being first used by Yoshida et al. [3]. In 2003, Notohara and coworkers described two types of AIP: Lymphoplasmacytic Sclerosing Pancreatitis (LPSP) termed “type 1 AIP” and idiopathic duct-centric chronic pancreatitis with Granulocyte Epithelial Lesion (GEL) termed “type 2 AIP” [1] (Table 1).
Highlights
Autoimmune Pancreatitis (AIP) is an emerging clinical entity found in 4.6-6 percent of patients with chronic pancreatitis [1]
It was first reported as an idiopathic chronic pancreatitis associated with hypergammaglobulinemia by Sarles et al [2], with the term AIP being first used by Yoshida et al [3]
Similar pathologic features were found in affected organs, and type 1 AIP began to be recognized as the pancreatic manifestation of a systemic autoinflammatory syndrome known as Immunoglobulin G4 (IgG4)-related disease [5]
Summary
Autoimmune Pancreatitis (AIP) is an emerging clinical entity found in 4.6-6 percent of patients with chronic pancreatitis [1]. It has been observed that T helper 1 (Th1) cells predominate over T helper 2 (Th2) cells in the peripheral blood while Th2 cells dominate over Th1 within the involved organs, and circulating levels of regulatory T cells (T-regs) are increased while naïve T-reg cells are decreased This has led some researchers to suggest a biphasic mechanism by which cytokines produced by Th1 cells induce AIP, and Th2 cytokines contribute to disease progression [6]. There is neutrophilic infiltration and granulocytic lesions that damage the duct epithelium itself, but no obliterative phlebitis or IgG4-positive plasma cells It tends to be associated with inflammatory bowel disease, but no other extra-pancreatic manifestations have been observed with this type of AIP [6]. MRI of our patient with AIP type 1 demonstrates diffuse swelling of pancreatic body and tail which resolved with a 2 month course of steroids (Figure 1A and 1B)
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