title: Raised numbers of IgG4-positive plasma cells are a common histopathological finding in orbital xanthogranulomatous disease.
abstract: OBJECTIVE
  To determine the relation of orbital xanthogranuloma with IgG4-related disease.
  
  
  METHODS
  Retrospective consecutive case series over a period of 25 years. We searched our charts for histologically confirmed orbital xanthogranuloma. Patient files were reviewed for clinical and follow up data including presence or absence of systemic non-ophthalmic manifestations of IgG4 related disease. Slides were re-examined and histopathological classification was re-assessed. Sixteen cases of orbital xanthogranuloma were evaluated. Immunohistochemical stains for IgG and IgG4 were performed. Positive immunohistochemical staining required increased IgG4-positive plasma cells in the involved tissues scored as >50 per high-power field, with IgG4/IgG ratio >0.40.
  
  
  RESULTS
  According to the criteria described above 8/16 (50%) cases showed increased numbers of IgG4-positive plasma cells in the specimens. Two of these patients may have had signs of systemic disease.
  
  
  CONCLUSIONS
  Raised numbers of IgG4-positive plasma cells are a common finding in histopathological specimens of xanthogranulomatous disease of the orbit and are often not indicative for IgG4 related systemic disease.
authors.affiliation: Department of Pathology .
authors.forename: Robert M
authors.initials: RM
authors.lastname: Verdijk
authors.forename: Pegah
authors.initials: P
authors.lastname: Heidari
authors.forename: Roel
authors.initials: R
authors.lastname: Verschooten
authors.forename: Paul L
authors.initials: PL
authors.lastname: van Daele
authors.forename: Huib J
authors.initials: HJ
authors.lastname: Simonsz
authors.forename: Dion
authors.initials: D
authors.lastname: Paridaens
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D006099:Granuloma / Q000097:blood / Q000175:diagnosis*; D006801:Humans; D007124:Immunoenzyme Techniques; D007074:Immunoglobulin G / Q000097:blood*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008206:Lymphatic Diseases / Q000097:blood / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009916:Orbital Diseases / Q000097:blood / Q000175:diagnosis*; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*; D012189:Retrospective Studies; D014973:Xanthomatosis / Q000097:blood / Q000175:diagnosis*
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents