June 2023
Volume 64, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2023
Ophthalmic Manifestations of IgG4-Related Disease
Author Affiliations & Notes
  • Carisa Bohnak
    Ophthalmology, Vanderbilt Eye Institute, Nashville, Tennessee, United States
  • Louise Mawn
    Ophthalmology, Vanderbilt Eye Institute, Nashville, Tennessee, United States
  • Hannah Harmsen
    Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Anthony Brent Daniels
    Ophthalmology, Vanderbilt Eye Institute, Nashville, Tennessee, United States
  • Footnotes
    Commercial Relationships   Carisa Bohnak None; Louise Mawn None; Hannah Harmsen None; Anthony Daniels None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2023, Vol.64, 3612. doi:
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      Carisa Bohnak, Louise Mawn, Hannah Harmsen, Anthony Brent Daniels; Ophthalmic Manifestations of IgG4-Related Disease. Invest. Ophthalmol. Vis. Sci. 2023;64(8):3612.

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

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Purpose : IgG4-Related Disease is a rare, heterogenous, fibro-inflammatory condition characterized by a dense lymphoplasmacytic infiltration with a high number of IgG4-bearing plasma cells. Ocular manifestations are not uncommon and can contribute substantially to disease morbidity. Most of what we know about IgG4-Related Disease (IgG4-ROD) is based on case reports or small studies, with the largest study describing 40 patients. The purpose of this study is to describe the ocular manifestations of this disease in both adults and children at a large, single institution to better characterize the disease, its course, and to help guide therapy.

Methods : A retrospective case series of patients with IgG4-ROD who have presented with ocular manifestations over the last 50 years. Patients were identified using ICD-10 billing codes and review of pathology records. Since other conditions can have similar presentations, only eyes in whom a confirmatory tissue diagnosis had been performed were included. Continuous variables were summarized by mean ± standard deviation and categorical variables by count and percentage.

Results : 20 patients were identified. The average patient age was 51 (±17). 55% of patients were female and 45% were male. The most common presenting ocular symptom was eyelid edema (55%). The most common ocular manifestation was orbital pseudotumor present in 90% of patients. Other ocular manifestations included scleritis (15%), iritis (5%), conjunctivitis (10%), and intraocular inflammation (5%). 90% of patients were initially managed as another disease entity including meningioma, chronic sinusitis, and lymphoma. 85% of patients required treatment with an immunomodulatory agent within their course. The average patient follow-up was 63 months ±68 (range 3-266). The average presenting visual acuity was 20/40 (range 20/20-LP) and the average final visual acuity was 20/60 (range 20/20-NLP). Only two patients lost >2 lines of vision during this follow-up period.

Conclusions : IgG4-ROD is a heterogenous condition that presents with both orbital and ocular findings, with inflammatory orbital pseudotumor predominating. It can masquerade as other conditions, and is often initially misdiagnosed, and so familiarity with this rare condition and a high index of suspicion should be maintained. Immunomodulatory therapy is the cornerstone of treatment and can help preserve vision in most patients. This represents one of the largest series of IgG4-ROD to date.

This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.


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