Mikiko Ohara

A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease

Mikuliczs disease (MD) has been included within the diagnosis of primary Sjögrens syndrome (SS), but it represents a unique condition involving persistent enlargement of the lacrimal and salivary glands characterized by few autoimmune reactions and good responsiveness to glucocorticoids, leading to the recovery of gland function. Mikuliczs disease was recently reported to be associated with elevated immunoglobulin G4 (IgG4) concentrations in the serum and prominent infiltration of plasmacytes expressing IgG4 into the lacrimal and salivary glands. The following features were used for diagnosis: (1) visual confirmation of symmetrical and persistent swelling in more than two lacrimal and major salivary glands; (2) prominent mononuclear cell infiltration of lacrimal and salivary glands; and (3) exclusion of other diseases that present with glandular swelling, such as sarcoidosis and lymphoproliferative disease. These features are not observed in most SS cases. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedels thyroiditis, all of which show IgG4 involvement in their pathogenesis. Mikuliczs disease thus differs from SS and may be a systemic IgG4-related plasmacytic disease.

Elevated IgG4 concentrations in serum of patients with Mikulicz's disease

Mikuliczs disease has recently been included within primary Sjögrens syndrome. It is a unique condition involving enlargement of the lacrimal and salivary glands, characterized by few autoimmune reactions. It is responsive to glucocorticoid treatment. Analysis of IgG fractions was performed in patients with Mikuliczs disease in order to determine the differences between Mikuliczs disease and Sjögrens syndrome. The study showed that serum IgG4 concentrations are elevated in patients with Mikuliczs disease, but not in those with Sjögrens syndrome.

A case of Mikulicz's disease (IgG4‐related plasmacytic disease) complicated by autoimmune hypophysitis

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