Eosinophilic pancreatitis with serum IgG4-positivity, potentially associated with autoimmune pancreatitis

A 70-year-old man presented with unprovoked weight loss and intermittent left upper quadrant tenderness for one-and-a-half month. Computed tomography revealed irregular cystic lesions in the pancreatic tail. Endoscopic ultrasonography (EUS) revealed pancreatic tail enlargement and giant, thick-walled cysts surrounding the pancreatic tail with no connection with the pancreatic duct. Endoscopic ultrasonography-guided fine-needle aspiration was performed on the enlarged pancreatic parenchyma and thickened cyst wall. Both biopsies showed hyper eosinophilia and few plasma cells. Endoscopic retrograde pancreatography revealed an irregular narrowing of the main pancreatic duct. Pancreatic juice cytology revealed substantial eosinophilia. Blood sampling showed an elevated eosinophil count and immunoglobulin G4 (IgG4) and immunoglobulin E (IgE) levels at the initial examination. We considered the patient to have eosinophilic pancreatitis (EP) with autoimmune pancreatitis, or alternately, EP with IgG4-related retroperitoneal fibrosis (RPF). Upon prednisolone administration, the abdominal pain improved, the peripheral blood eosinophil count decreased to zero, IgG4 and IgE levels decreased, pancreatic enlargement improved, and the cystic lesions disappeared. The condition did not recur within the following 3 years. Both EP and EP with IgG4-related RPF are rare etiologies of pancreatitis, and this case is very instructive.