An unusual case of autoimmune pancreatitis presenting as pancreatic mass and obstructive jaundice: a case report and review of the literature

A 31-year-old Caucasian man presented to our hospital with epigastric pain, jaundice and weight loss. His CA 19-9 level was elevated, and computed tomography and endoscopic ultrasound revealed a pancreatic head mass abutting the portal vein. Endoscopic retrograde cholangiopancreaticography showed narrowing of the biliary duct and poor visualization of the pancreatic duct. Fine-needle aspiration biopsy revealed atypical ductal epithelial cells, which raised clinical suspicion of adenocarcinoma. Because of the patient's unusual age for the onset of pancreatic cancer and the acuity of his symptoms, he was referred to a tertiary care center for further evaluation. His immunoglobulin G4 antibody level was 365 mg/dL, and repeat computed tomography showed features typical of autoimmune pancreatitis. The patient's symptoms resolved with corticosteroid therapy.Autoimmune pancreatitis is a rare disease with an excellent response to corticosteroid therapy. Its unique histological appearance and response to corticosteroid therapy can reduce unnecessary surgical procedures. A thorough evaluation by a multidisciplinary team is important in rendering the diagnosis of autoimmune pancreatitis.Autoimmune pancreatitis (AIP), also known as lymphoplasmacytic sclerosing pancreatitis, is a rare disease of the pancreas that is part of a systemic fibro-inflammatory syndrome complex. It is characterized by multi-organ immunoglobulin G4 (IgG4)-rich lymphoplasmacytic infiltration. The pancreas, biliary tree, salivary glands, retroperitoneum, lymph nodes and kidneys can be involved in this systemic fibrotic condition [1]. Although first described in 1961, it was not until 1995 that this disease was named "autoimmune pancreatitis" [2,3].While the exact prevalence of AIP is unknown, the estimated prevalence is between 4.6% and 6% in patients with chronic pancreatitis and 11% in patients undergoing pancreatic resection for suspected malignancy [4,5]. There is a 2:1 male predominance. The patient's