A primary Sjogren's syndrome patient with marked swelling of multiple exocrine glands and sclerosing pancreatitis

A 55-year-old man suffered from nasal obstruction, swelling of the salivary glands, and diplopia caused by markedly enlarged lacrimal glands. The diagnosis of primary Sjogren's syndrome was made by a positive Schirmer's test and nasal mucosal biopsy with severe lymphocyte infiltration. He was also found to have swelling of the whole pancreas and increased wall thickness in the common bile duct and the gall bladder. His serum was positive for an anticarbonic anhydrase II antibody. Since carbonic anhydrase II is present in the ductal cells of various exocrine organs, this autoantibody is considered to be related to the pathogenesis of primary Sjogren's syndrome with a marked swelling of multiple exocrine organs.