Clinicopathological characteristics of Mikulicz's disease and Küttner's tumor

Abstract

Mikulicz's disease (MD) has been considered part of primary Sjogren's syndrome (SS) since Morgan's report in 1953. However, MD shows a unique condition involving persistent swelling of the lachrymal and salivary glands characterized by good responsiveness to glucocorticoids, leading to the recovery of gland function. Recently, it has been revealed that MD patients show elevated serum immunoglobulin G4 (IgG4) concentrations and prominent infiltration of IgG4-positive plasma cells. The complications of MD include autoimmune pancreatitis, retroperotoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Thus, MD is a systemic IgG4-plasmacytic disease. On the other hand, Kuttner's tumor (KT) is chronic sclerosing sialadenitis that presents with asymmetrical firm swelling of the submandibular glands. Immunohistochemical analyses have revealed that a proportion of IgG4-positive plasma cells was also elevated in KT. MD and KT differ from SS and are thought to possibly form a singular systemic IgG4-related plasmacytic disease.