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Bilateral IgG4-related ophthalmic disease: a strong indication for systemic imaging
  1. Albert Wu1,
  2. Nicholas H Andrew1,
  3. Alan A McNab2,
  4. Dinesh Selva1
  1. 1South Australian Institute of Ophthalmology, University of Adelaide, Adelaide, Australia
  2. 2Centre for Eye Research Australia, University of Melbourne, Melbourne, Australia
  1. Correspondence to Albert Wu, South Australian Institute of Ophthalmology, Level 8, East Wing, Royal Adelaide Hospital, Adelaide, SA 5000, Australia; albert.wu3{at}


Background/aims 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.

  • Immunology
  • Inflammation
  • Orbit

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