title: Bilateral IgG4-related ophthalmic disease: a strong indication for systemic imaging.
abstract: OBJECTIVE
  To investigate whether bilateral or unilateral IgG4-related ophthalmic disease (IgG4-ROD) is associated with extra-ophthalmic IgG4-related disease (IgG4-RD).
  
  
  METHODS
  Twin-centre retrospective observational case series of biopsy-confirmed IgG4-ROD. Clinical and radiology data were reviewed for laterality of IgG4-ROD and presence of extra-ophthalmic disease. The literature was reviewed for case series of IgG4-ROD.
  
  
  RESULTS
  40 IgG4-ROD cases were identified, with median follow-up of 36 months. At diagnosis of IgG4-ROD, all cases were screened for extra-ophthalmic disease with physical examination and blood testing. Systemic imaging was performed in 20 (50%) cases due to clinical suspicion of extra-ophthalmic disease. Of the 21 unilateral IgG4-ROD cases, 3 (14%) had extra-ophthalmic involvement. Of the 19 bilateral cases, 15 (79%) had extra-ophthalmic involvement. Extra-ophthalmic involvement was strongly associated with bilateral IgG4-ROD (p<0.001). On pooling our data (n=40) with previously published cases (n=142), the association remained strong (p<0.001).
  
  
  CONCLUSIONS
  Bilateral IgG4-ROD is strongly associated with extra-ophthalmic IgG4-RD. We recommend that imaging of the neck, chest, abdomen and pelvis be performed for all bilateral cases. Systemic imaging should also be considered in unilateral cases as a significant proportion of these patients will also have extra-ophthalmic disease.
authors.affiliation: South Australian Institute of Ophthalmology, University of Adelaide, Adelaide, Australia.
authors.forename: Albert
authors.initials: A
authors.lastname: Wu
authors.affiliation: South Australian Institute of Ophthalmology, University of Adelaide, Adelaide, Australia.
authors.forename: Nicholas H
authors.initials: NH
authors.lastname: Andrew
authors.affiliation: Centre for Eye Research Australia, University of Melbourne, Melbourne, Australia.
authors.forename: Alan A
authors.initials: AA
authors.lastname: McNab
authors.affiliation: South Australian Institute of Ophthalmology, University of Adelaide, Adelaide, Australia.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Selva
mesh_terms: D000888:Antibodies, Anti-Idiotypic / Q000097:blood / Q000276:immunology*; D001327:Autoimmune Diseases; D001706:Biopsy; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging / Q000379:methods*; D005128:Eye Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D008297:Male; D012189:Retrospective Studies
chemical_list: D000888:Antibodies, Anti-Idiotypic; D007074:Immunoglobulin G
keywords: Immunology; Inflammation; Orbit