title: High prevalence of IgG4-related lymphoplasmacytic infiltrative disorder in 25 patients with orbital inflammation: a retrospective case series.
abstract: OBJECTIVE
  To evaluate retrospectively the prevalence of positive IgG4-immunostaining in orbital tissue of patients with idiopathic orbital inflammation and to compare the clinical, radiographic and pathologic features among patients with and without IgG4-positive cells.
  
  
  METHODS
  25 patients with biopsy-proven idiopathic orbital inflammation examined from January 2006 through December 2011 were included. Immunohistochemistry with IgG and IgG4 immunostaining from biopsy specimens of all patients was performed. Tissue with more than 10 IgG4-positive plasma cells per high-power field and with a ratio of IgG4+/IgG+ plasma cells of more than 40% was scored as positive. Histopathologic features, demographic and clinical data, radiologic findings, treatment and follow-up information for each patient were analysed.
  
  
  RESULTS
  Immunohistochemical staining showed 10 cases (40%) were IgG4 positive. The symptoms and signs included eyelid or periocular swelling/mass in all, pain (3/10), extraocular muscle restriction (3/10), proptosis (5/10) and/or decreased vision (4/10). Demographic and clinical findings of these patients did not differ from those with IgG4-negative cells. The presence of positive IgG4-immunostaining in orbital tissue was significantly associated with characteristic pathological features (more background fibrosis, lymphoid hyperplasia, plasma cells and phlebitis).
  
  
  CONCLUSIONS
  Finally, 40% of patients with biopsy-proven orbital inflammation were classified as IgG4-RD, with typical histological features, but without specific clinical or radiological findings.
authors.affiliation: Department of Neurology, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France. rdeschamps@fo-rothschild.fr
authors.forename: Romain
authors.initials: R
authors.lastname: Deschamps
authors.forename: Lydia
authors.initials: L
authors.lastname: Deschamps
authors.forename: Raphael
authors.initials: R
authors.lastname: Depaz
authors.forename: Sophie
authors.initials: S
authors.lastname: Coffin-Pichonnet
authors.forename: Georges
authors.initials: G
authors.lastname: Belange
authors.forename: Pierre Vincent
authors.initials: PV
authors.lastname: Jacomet
authors.forename: Catherine
authors.initials: C
authors.lastname: Vignal
authors.forename: Paul
authors.initials: P
authors.lastname: Benillouche
authors.forename: Marie Laure
authors.initials: ML
authors.lastname: Herdan
authors.forename: Marc
authors.initials: M
authors.lastname: Putterman
authors.forename: Anne
authors.initials: A
authors.lastname: Couvelard
authors.forename: Olivier
authors.initials: O
authors.lastname: Gout
authors.forename: Olivier
authors.initials: O
authors.lastname: Galatoire
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005094:Exophthalmos / Q000175:diagnosis; D058447:Eye Pain / Q000175:diagnosis; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007150:Immunohistochemistry; D008206:Lymphatic Diseases / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015835:Ocular Motility Disorders / Q000175:diagnosis; D016727:Orbital Pseudotumor / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*; D015995:Prevalence; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014786:Vision Disorders / Q000175:diagnosis; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G
keywords: Epidemiology; Inflammation; Orbit