A Case of Type 1 Autoimmune Pancreatitis

摘要

Autoimmune pancreatitis (AIP) is a form of pancreatitis with clinical, serological and histological features of an autoimmune process. This is the case of a 32-year-old diabetic man who had been suffering from painless jaundice with pale stools and tea-colored urine for 3 months prior to consultation. An endoscopic retrograde cholangiopancreatography revealed a proximal common bile duct stricture, and magnetic resonance cholangiopancreatography showed a bulky pancreatic head. His serum amylase level was mildly raised, and his serum IgG titer was markedly elevated. The serum alkaline phosphatase and conjugated biulirubin levels were high, suggesting an obstructive jaundice. A diagnosis of AIP was made and treated with steroids. He responded well to the steroid therapy. Introduction Autoimmune pancreatitis is a rare condition in contrast to other autoimmune disorders. It affects men more often than women (ratio 2:1) and up to 70–80% of patients present with painless jaundice (Buscarinia et al., 2010). Marked cachexia, inability to eat and narcotic-requiring pain are more suggestive of pancreatic cancer (Shimosegawa et al., 2011), on the contrary, but the case is considered autoimmune due to its dramatic response to steroid therapy. Currently, elevated serum IgG4 levels are considered to be the sole serological hallmark of AIP (Buscarinia et al., 2010). This is a case of autoimmune pancreatitis reported to Universiti Kebangsaan Malaysia Medical Centre (UKMMC) for diagnosis and management. Case Presentation This 32-year-old diabetic gentleman presented to UKMMC for diagnosis and management of autoimmune pancreatitis. The patient had suffered from painless jaundice for three months prior to hospitalization. The patient’s stool was pale with tea-colored urine. There were no symptoms of vomiting or anorexia and no alteration in bowel habits. He had no history of fever. He had no viral hepatitis and had not been admitted to a hospital for pancreatitis before. He was a long-term smoker and alcoholic. The first radiological investigation was performed by endoscopic retrograde cholangiopancreatography (ERCP) and revealed a proximal common bile duct stricture, possibly secondary to external compression. Following this, a magnetic resonance cholangiopancreatography was done, and we observed a bulky pancreatic head and a thickened gallbladder wall with a gallstone inside. The blood investigations were performed, and the results are presented in Table 1.