Type 1 Autoimmune Pancreatitis in a Young Male

Autoimmune pancreatitis (AIP) is a rare entity leading to inflammation of the pancreas. It can be broadly categorized into two types. Type 1 AIP is more common and primarily presents with jaundice. Less commonly it can also progress to multiorgan involvement. Here we report a case of a 19-year-old male who presented to us with complaints of abdominal pain and vomiting. His laboratory investigations showed raised serum amylase and lipase. A contrast-enhanced CT revealed a diffuse enlargement of the pancreas with internal low-density foci. Due to the repeated episodes of pancreatitis, the patient's blood was tested for serum IgG4 (immunoglobulin type G4) which was markedly elevated pointing toward the diagnosis of AIP. Endoscopic ultrasound (EUS) showed a sausage-shaped pancreas with hyper- and hypoechoic strands. EUS-guided fine needle aspiration cytology of the lymph nodes performed in the celiac region showed a mixed population of lymphoid cells. Based on all the workup, our patient was diagnosed as type 1 AIP. He was managed with steroids and his condition progressively improved. This case is clinically significant because of the close resemblance of AIP with other pancreatic disorders like neoplasm. A timely diagnosis can prevent the unnecessary performance of invasive procedures in these patients.