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.