The newly established concepts of Mikulicz's disease and Kuttner's tumor (chronic sialadenitis) are discussed. Mikulicz's disease has been included within the diagnosis of primary Sjogren's syndrome, but it represents a unique condition involving persistent enlargement of the lacrimal and salivary glands. Recently, elevated IgG4 concentration in serum and prominent infiltration by plasmacytes expressing IgG4 in the salivary glands was demonstrated in Mikulicz's disease. Mikulicz's disease

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... s from Sjogren's syndrome, and may be a systemic IgG4-related plasmacytic disease. Kuttner's tumor is a chronic sclerosing sialadenitis that presents with asymmetrical firm swelling of the submandibular glands. The histological characteristics of Kuttner's tumor are severe fibrosis, atrophic acinus, and mononuclear cell infiltration. Immunohistochemical evaluation demonstrated that the proportion of IgG4-positive plasmacytes was elevated in Kuttner's tumor similar to that in Mikulicz's disease. Therefore, it is possible that both Mikulicz's disease and Kuttner's tumor may involve a similar pathogenesis of IgG4-related disease as a form of autoimmune pancreatitis.