title: Clinical characteristics of Mikulicz's disease as an IgG4-related disease.
abstract: OBJECTIVE
  Mikulicz's disease (MD) was considered to be a subtype of Sjögren's syndrome (SS), based on histopathological similarities. However, recent studies have indicated that patients with MD show high serum IgG4 concentration, and suggested that MD is one of "IgG4-related disease" and distinguishable from SS. Therefore, we clinically and histopathologically examined the disease states of MD and SS in detail.
  
  
  METHODS
  Twenty patients with Mikulicz's disease and 18 with SS were comparatively studied to determine clinical characteristics in MD patients.
  
  
  RESULTS
  Sialography in MD patients did not show the "apple-tree sign" typically seen in SS. Serologically, high serum IgG4 levels but not anti-SS-A or anti-SS-B antibodies were observed in MD. SS showed lymphocytic infiltration of various subsets with atrophy or severe destruction of the acini, while MD showed selective infiltration of IgG4+ plasma cells with hyperplastic germinal centers and mild acini destruction. Corticosteroid treatment of MD reduced IgG and IgG4 levels and improved salivary function. A negative correlation between disease duration and increasing rate of salivary flow was observed in MD.
  
  
  CONCLUSIONS
  These results suggested that the pathogenesis of MD might be different from those of SS.
  
  
  CONCLUSIONS
  early diagnosis and treatment of MD is important for the improvement of salivary function.
authors.affiliation: Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
authors.forename: Masafumi
authors.initials: M
authors.lastname: Moriyama
authors.forename: Akihiko
authors.initials: A
authors.lastname: Tanaka
authors.forename: Takashi
authors.initials: T
authors.lastname: Maehara
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Ohyama
authors.forename: Mayumi
authors.initials: M
authors.lastname: Shimizu
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Nakashima
authors.forename: Jun-Nosuke
authors.initials: JN
authors.lastname: Hayashida
authors.forename: Shoichi
authors.initials: S
authors.lastname: Shinozaki
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Kubo
authors.forename: Sachiko
authors.initials: S
authors.lastname: Furukawa
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Kikuta
authors.forename: Seiji
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D012463:Saliva / Q000378:metabolism
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G