Vol.16 No.6


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


Motohisa Yamamoto1, Hiroki Takahashi1, Mikiko Ohara1, Chisako Suzuki1, Yasuyoshi Naishiro1, Hiroyuki Yamamoto1, Yasuhisa Shinomura1 and Kohzoh Imai2

(1) First Department of Internal Medicine, Sapporo Medical University School of Medicine, South 1, West 16, Chuo-ku, Sapporo 060-8543, Japan
(2) Sapporo Medical University, Sapporo, Japan

April 10, 2006


July 12, 2006


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Mikulicz’s disease (MD) has been included within the diagnosis of primary Sjogren’s 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. Mikulicz’s 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 mphoproliferative disease. These features are not observed in most SS cases. The complications of MD include utoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel’s thyroiditis, all of which show IgG4 involvement in their pathogenesis. Mikulicz’s disease thus differs from SS and may be a systemic IgG4-related plasmacytic disease.

Key words

Autoimmune pancreatitis, Glucocorticoid, Immunoglobulin G4, Mikulicz's disease, Sjögren's syndrome