July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
Ocular and orbital involvement in IgG4-related disease
Author Affiliations & Notes
  • Thomas Ness
    Eye Center, University of Freiburg, Freiburg, Germany
  • Moritz Daniel
    Eye Center, University of Freiburg, Freiburg, Germany
  • Wolf Lagreze
    Eye Center, University of Freiburg, Freiburg, Germany
  • Sonja Heinzelmann-Mink
    Eye Center, University of Freiburg, Freiburg, Germany
  • Footnotes
    Commercial Relationships   Thomas Ness, None; Moritz Daniel, None; Wolf Lagreze, None; Sonja Heinzelmann-Mink, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 6699. doi:
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    • Get Citation

      Thomas Ness, Moritz Daniel, Wolf Lagreze, Sonja Heinzelmann-Mink; Ocular and orbital involvement in IgG4-related disease. Invest. Ophthalmol. Vis. Sci. 2019;60(9):6699.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose : The IgG4-related disease (IgG4-RD) is a multi-organ disease characterized by an increase in IgG4 positive plasmocytes and elevated IgG4 serum level. The aim of our study was to analyze orbital and ocular manifestations of the aforementioned disease in a cohort at a German academic center.

Methods : Patients diagnosed with IgG4-RD were identified from the electronic database of the Eye Center, University of Freiburg. The following data were analyzed: mean age, gender, orbital or ocular manifestation, laterality, therapy.

Results : We identified 11 patients with ocular/orbital manifestation of IgG4-RD. Seven patients showed orbital manifestation, while 4 patients showed ocular manifestation. Most common orbital involvements were orbital pseudotumor, inflammation of the lacrimal gland, and orbital granuloma, while most common ocular involvements were scleritis, anterior uveitis, and retinal vasculitis. In contrast to orbital manifestation, ocular manifestation tended to be bilateral. Patients with ocular involvement were younger compared to patients with orbital involvement (44 years : 61 years (mean)). There was a predominance in male patients (7:4). All patients who received rituximab treatment responded well. Systemic manifestations included generalized lymphadenopathy, autoimmune pancreatitis, interstitial nephritis, thrombophlebitis, and intestinal occlusion.

Conclusions : IgG4-RD causes not only orbital, but can also lead to ocular pathology. IgG4-RD should be considered in patients with uveitis and systemic symptoms. New antibody therapies provide a high chance of remission.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.


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