title: Clinicopathologic features of orbital immunoglobulin G4-related disease (IgG4-RD): a case series and literature review.
abstract: BACKGROUND
  Involvement of orbital structures by immunoglobulin G4-related disease (IgG4-RD) is not uncommon. We conducted this study to evaluate the clinicopathologic features of orbital IgG4-RD.
  
  
  METHODS
  This was a retrospective, clinicopathologic study. Clinical records, light microscopic features, results of immunostaining with IgG & IgG4 and laboratory findings were reviewed in 16 patients diagnosed with orbital IgG4-RD.
  
  
  RESULTS
  Eleven patients had a bilateral disease, and the lacrimal gland was involved in 14. Dense sclerosis, plasma cell aggregates and dense lymphoplasmacytic infiltrate were seen in all patients. Serum IgG4 titre was elevated in 12 patients. Nine patients responded completely to glucocorticoid treatment. Five patients had a relapse on discontinuation of treatment.
  
  
  CONCLUSIONS
  Orbital IgG4-RD is a distinct clinicopathologic entity requiring increased awareness and needs to be differentiated from other orbital lymphoproliferative lesions.
authors.affiliation: National Reporting Centre for Ophthalmic Pathology (NRCOP), Centre For Sight, Ashoka Capitol Building, Banjara Hills, Road No.2, Hyderabad, 500034, India, kaustubh.m@hotmail.com.
authors.forename: Kaustubh
authors.initials: K
authors.lastname: Mulay
authors.forename: Ekta
authors.initials: E
authors.lastname: Aggarwal
authors.forename: Santosh G
authors.initials: SG
authors.lastname: Honavar
mesh_terms: D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007262:Infusions, Intravenous; D007766:Lacrimal Apparatus Diseases / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D055622:Orbital Myositis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D010265:Paraproteinemias / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D010950:Plasma Cells / Q000473:pathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D008775:Methylprednisolone