Autoimmune pancreatitis associated with various extrapancreatic lesions during a long-term clinical course successfully treated with azathioprine and corticosteroid maintenance therapy.

Autoimmune pancreatitis (AIP) is often associated with systemic extrapancreatic lesions, and the concept of IgG4-related autoimmune disease has been proposed. We report a 62-year-old woman with AIP associated with various extrapancreatic lesions during a long-term clinical course. These extrapancreatic lesions occurred after the withdrawal of steroid therapy. Sclerosing sialadenitis occurred before AIP, whereas autoimmune sensorineural hearing loss, allergic purpura and endocapillary proliferative glomerulonephritis occurred after AIP. However, no relapse was observed for 5 years after the start of maintenance therapy with prednisolone and azathioprine.