A sixties man presented with a left neck mass and upper abdominal pain. The left parotid and submandibular glands were enlarged, and blood tests showed amylase 525IU/l, IgG 4,023mg/dl, and IgG4 3,120mg/dl. Abdominal ultrasonography, CT scan, and MRI revealed an enlarged pancreas, with a capsule-like rim, and multiple pancreatic cysts. Histopathological examination of tissue obtained by EUS-FNAB demonstrated mottled fibrosis, invasion of plasma cells, storiform fibrosis, and obliterative phlebitis. Based on these findings, the patient was diagnosed with type 1 autoimmune pancreatitis. Twelve weeks after starting administration of prednisolone, the pancreatic enlargement had resolved and the pancreatic cysts had almost disappeared. This is a rare case of multiple cysts associated with autoimmune pancreatitis that responded to steroid treatment.
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