Introduction Autoimmune pancreatitis is a rare paediatric condition with management driven by adult guidelines. However, case reports suggest that the paediatric disease presents differently with few children presenting with elevated IgG4 levels. Management is also unclear with limited evidence to suggest any management strategy superior to another or the long-term outcomes of paediatric patients.

Aim To present a case of possible autoimmune pancreatitis, presenting with obstructive jaundice, managed without steroids.

Subject: A 12-year-old Caucasian male presented with a week of pruritus and bruising with a 48-hour history of diarrhoea and jaundice. There was no history of abdominal pain, weight loss or fatigue. He was previously fit and well with no significant family history. Examination revealed petechiae and ecchymosis but no positive abdominal findings.

Results At presentation bloods showed bilirubin 277 umol/L (201 umol/L conjugated), alanine transaminase 88 unit/L, alkaline phosphatase 802 unit/L, gamma glutamyltransferase 79 unit/L and international normalised ratio 0.9. Amylase and lipase were normal throughout. Abdominal ultrasound at the presenting hospital revealed a 2.5 cm mass at the head of the pancreas with a dilated gallbladder.

The child underwent a MRCP which showed a 3 cm mass at the pancreatic head with marked intra and extrahepatic biliary dilatation. The pancreatic duct was not visible in the pancreatic head. Endoscopic ultrasonography confirmed a diffusely abnormal pancreas with no pancreatic duct dilatation. There was no discrete mass visible. An ERCP revealed a smooth single stenosis of 20 mm in the distal and mid common bile duct with pre-stenotic dilatation. A stent was inserted.

Autoantibodies were normal, as were immunoglobulins, including IgG4. Hepatitis serology and alpha-fetoprotein were also normal. A pancreatic biopsy was taken which showed mild chronic inflammation, minor acinar atrophy with intralobular oedema and some fibrosis. Immunohistochemistry for IgG4 disease was negative.

Maximal bilirubin was 334, alkaline phosphatase 869 and alanine transaminase 81 prior to stent insertion. These were falling prior to stent insertion however fell rapidly post insertion and were within normal limits 4 weeks post stent insertion. The patient received no further medical management and is due for repeat imaging prior to proposed stent removal in the next few weeks.

Summary We present a 12-year-old boy who presented with typical radiological findings of presumed autoimmune pancreatitis. He was managed endoscopically with stent insertion and achieved a rapid resolution in symptoms and biochemical markers. Autoimmune screening was negative and there were no positive diagnostic findings with regard to IgG4. The child did not receive steroid therapy and is awaiting repeat imaging prior to proposed stent removal.

Conclusion Autoimmune pancreatitis is a rare condition in children and management is often extrapolated from adult guidelines. Collaborative working is required for the creation of paediatric guidelines to guide further management of this rare condition.