Severe obstructive jaundice in a young patient with IgG4-negative autoimmun pancreatitis – treatment without invasive procedures

Introduction: Autoimmun pancreatitis should be a differenzial diagnosis from other form of chronic pancreatitis and pancreas malignancies in jaundice, abdominal pain, weight loss with pancreas enlargement, narrowing of the bile duct and abnormal immunserological tests. Here we report a 28-year-old previously healthy male with ANA, IgG4- negative autoimmun pancreatitis according to the findings of serum biochemistry, CT and ERCP. Our patient presented with a 1 month history of obstructive jaundice, epigastrial pain, weight loss after using itraconazol for skin mycosis. His serum bilirubin and ALP were highly elevated on admission. The transaminase levels were only mildly elevated and serum amylase was in normal range. Abdominal US showed a dilated common bile duct (CBD) and intrahepatic biliary radicals, the head and body of the pancreas was bulky. There were no gallstones, CBD stones or any mass lesions in the CBD. The CT scan of the abdomen showed the enlargement of the head and body with dilated intra-and extrahepatic bile ducts. Hypergammaglobulinaemia, autoantibodies and elevated serum IgG4 levels were not detected. CA 19–9, CEA were in normal range. Fine needle aspiration of the pancreas was done, malignancy was not observed. During ERCP diffuse narrowing of pancreatic duct and intrapancreatic part of the CBD were found, papillotomy and biliary drainage were not performed. Biopsy was taken from the papilla of Vateri, IgG4 positive cells were not detected. Diabetes mellitus appeared, needing just diet. Oral prednisolon therapy was started. After a few weeks, the diffuse enlargement of the pancreas on CT reverted to normal. Dilation of the bile ducts and elevated liver enzymes normalized. Paralelly clinical improvement was observed, abdominal pain, weigt loss and jaundice resolved, serum blood glucose levels normalized on diet. Conclusion: AIP was diagnosed based on clinical symptoms and ERCP pictures. Early initiation of corticostreoid therapy may prevent invasive endoscopic procedures in the management of obstructive jaundice.