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Recent histological and clinical studies have suggested the existence of 2 distinct types of autoimmune pancreatitis (AIP): type 1 AIP related to IgG4, exhibiting lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP related to granulocyte epithelial lesions (GELs), exhibiting idiopathic duct-centric chronic pancreatitis (IDCP). We herein present a case of type 1 AIP with histologically proven LPSP with GELs. This patient had neither serum IgG4 elevation nor MPD narrowing. In this case, the clinically and histologically atypical findings for type 1 AIP are intriguing.

Autoimmune Pancreatitis with Histologically Proven Lymphoplasmacytic Sclerosing Pancreatitis with Granulocytic Epithelial Lesions