A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease.
Mikulicz's disease (MD) has been included within the diagnosis of primary Sjögren'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 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 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.
Yamamoto
Motohisa
M
First Department of Internal Medicine, Sapporo Medical University School of Medicine, South 1, West 16, Chuo-ku, Sapporo 060-8543, Japan. mocha@cocoa.plala.or.jp
Takahashi
Hiroki
H
Ohara
Mikiko
M
Suzuki
Chisako
C
Naishiro
Yasuyoshi
Y
Yamamoto
Hiroyuki
H
Shinomura
Yasuhisa
Y
Imai
Kohzoh
K
D000328:Adult; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000266:history / Q000276:immunology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D049672:History, 19th Century; D049673:History, 20th Century; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007765:Lacrimal Apparatus / Q000276:immunology / Q000473:pathology; D007963:Leukocytes, Mononuclear / Q000473:pathology; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000266:history / Q000276:immunology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D012219:Rheumatology / Q000266:history / Q000379:methods*; D012469:Salivary Glands / Q000276:immunology / Q000473:pathology; D012859:Sjogren's Syndrome / Q000276:immunology / Q000473:pathology
D005938:Glucocorticoids; D007074:Immunoglobulin G