title: [Clinical research of immunoglobin 4-related Mikulicz's disease].
abstract: OBJECTIVE
  To explore the clinical features of immunoglobin (IgG4)-related Mikulicz's disease (MD).
  
  
  METHODS
  Since December 2010, a prospective cohort study of IgG4 related disease (IgG4RD) was performed our hospital. A total of 42 IgG4RD patients were recruited along with 18 MD patients. Their clinical, laboratory and histopathological features and response to treatment were analyzed.
  
  
  RESULTS
  The 18 MD patients accounted for 42.9% of IgG4RD. There were 10 males and 8 females with a mean age of (48 ± 14) years. The mean follow-up period was 8.18 months. All of them had an involvement of salivary and lacrimal glands. Fifteen cases suffered other organs' damage, including autoimmune pancreatitis (n = 7), sclerosing cholangitis (n = 4) and lymph nodes (n = 6). And 10 patients complained of a history of allergies. All serum levels of IgG4 subclass significantly increased. Histological examinations in 16 patients revealed massive infiltration of lymphocytes and IgG4 positive plasma cells with obvious tissue fibrosis. Glucocorticoid and immunosuppressive therapies were effective for MD.
  
  
  CONCLUSIONS
  As an IgG4-related systemic disease involving predominantly lacrimal and salivary glands without autoantibodies, MD is treated optimally with a combination therapy of glucocorticoid and immunosuppressive drugs.
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100032, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Lin
authors.forename: Hua
authors.initials: H
authors.lastname: Chen
authors.forename: Qing-jun
authors.initials: QJ
authors.lastname: Wu
authors.forename: Wen-jie
authors.initials: WJ
authors.lastname: Zheng
authors.forename: Qun
authors.initials: Q
authors.lastname: Shi
authors.forename: Xiao-mei
authors.initials: XM
authors.lastname: Leng
authors.forename: Dong
authors.initials: D
authors.lastname: Xu
authors.forename: Min
authors.initials: M
authors.lastname: Shen
authors.forename: Wen
authors.initials: W
authors.lastname: Zhang
authors.forename: Yan
authors.initials: Y
authors.lastname: Zhao
authors.forename: Xiao-feng
authors.initials: XF
authors.lastname: Zeng
authors.forename: Feng-chun
authors.initials: FC
authors.lastname: Zhang
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007765:Lacrimal Apparatus / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D011446:Prospective Studies; D012469:Salivary Glands / Q000503:physiopathology
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G