title: A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease.
abstract: 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.
authors.affiliation: 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
authors.forename: Motohisa
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Hiroki
authors.initials: H
authors.lastname: Takahashi
authors.forename: Mikiko
authors.initials: M
authors.lastname: Ohara
authors.forename: Chisako
authors.initials: C
authors.lastname: Suzuki
authors.forename: Yasuyoshi
authors.initials: Y
authors.lastname: Naishiro
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Yamamoto
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Shinomura
authors.forename: Kohzoh
authors.initials: K
authors.lastname: Imai
mesh_terms: 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
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G