title: An analysis of IgG4-related disease (IgG4-RD) among idiopathic orbital inflammations and benign lymphoid hyperplasias using two consensus-based diagnostic criteria for IgG4-RD.
abstract: OBJECTIVE
  To determine the proportion of idiopathic orbital inflammation (IOI) and orbital benign lymphoid hyperplasia (OBLH) accounted for by immunoglobulin (Ig)G4-related orbital disease (IgG4-ROD) using the comprehensive diagnostic criteria for IgG4-related disease published by Umehara et al and the consensus diagnostic criteria published by Deshpande et al. Secondary aims were to compare the histological and clinical features of IgG4-ROD and non-IgG4-ROD cases, and to compare IgG4-ROD cases diagnosed using the comprehensive diagnostic criteria with those diagnosed using the consensus diagnostic criteria.
  
  
  METHODS
  A retrospective histopathological review and clinical case series. 55 cases of biopsy-confirmed non-granulomatous IOI and 10 cases of biopsy-confirmed OBLH were included. The intensity of sclerosis, lymphoplasmacytic infiltration and eosinophilic infiltration was graded from 0 to 3+ using a standardised and validated scoring system.
  
  
  RESULTS
  IgG4-ROD accounted for 50% and 40% of cases originally diagnosed as OBLH and 23.6% and 5.4% of cases originally diagnosed as IOI, using the comprehensive diagnostic criteria and the consensus diagnostic criteria, respectively. IgG4-ROD cases had numerous significant histological differences, but relatively few significant clinical differences, from non-IgG4-ROD cases. Compared with the comprehensive diagnostic criteria, the consensus diagnostic criteria identified a group of IgG4-ROD cases with a slightly higher ratio of IgG4+ to IgG+ (p=0.01) and a slightly longer duration of symptoms (p=0.02).
  
  
  CONCLUSIONS
  This is the largest review of IgG4 staining among biopsy-confirmed IOI and OBLH. IgG4-ROD accounted for a substantial proportion of OBLH. The prevalence among cases of IOI was considerably reduced when the consensus diagnostic criteria were used in place of the comprehensive diagnostic criteria.
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Adelaide, Adelaide, South Australia, Australia.
authors.forename: Nicholas H
authors.initials: NH
authors.lastname: Andrew
authors.affiliation: Department of Surgical Pathology, SA Pathology, Adelaide, South Australia, Australia.
authors.forename: Nicole
authors.initials: N
authors.lastname: Sladden
authors.affiliation: Department of Surgical Pathology, SA Pathology, Adelaide, South Australia, Australia.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Kearney
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Adelaide, Adelaide, South Australia, Australia.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Selva
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D032921:Consensus; D005260:Female; D005355:Fibrosis; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009916:Orbital Diseases / Q000175:diagnosis* / Q000276:immunology; D016727:Orbital Pseudotumor / Q000175:diagnosis* / Q000276:immunology; D010950:Plasma Cells / Q000473:pathology; D019310:Pseudolymphoma / Q000175:diagnosis* / Q000276:immunology; D012189:Retrospective Studies
chemical_list: D007074:Immunoglobulin G
keywords: Inflammation; Lacrimal gland; Orbit; Pathology