IgG4 Negative Sclerosing Cholangitis Associated with Autoimmune Pancreatitis

BACKGROUND: Sclerosing pancreatitis is an autoimmune condition characterized by periductal lymphocytic infiltration on histology, and elevated serum auto-antibodies and IgG4. Bile duct involvement is often associated with sclerosing pancreatitis but it is rarely the dominant feature. CASE REPORT: We report a patient with dominant biliary stricture and obstructive jaundice associated with IgG4 negative autoimmune pancreatitis. Due to uncertainties regarding the preoperative diagnosis, the patient underwent bilio-enteric bypass with an intraoperative pancreatic and bile duct biopsy. Post operatively, due to disease recurrence, the patient was started on steroids with consequent remission of the disease. CONCLUSION: Sclerosing pancreatitis may sometimes present with biliary stricture due to bile duct involvement due to the inflammatory process. The condition is often recognized after surgical exploration for a suspected malignancy. Preoperative diagnosis permits treatment with steroids and endoscopic biliary decompression, and avoids unnecessary surgery.