June 2021
Volume 62, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2021
A Unique Ophthalmic Presentation Leading to a Diagnostic Dilemma in Wegener’s Granulomatosis
Author Affiliations & Notes
  • Kaylin Arpaia
    Howerton Eye Clinic, Austin, Texas, United States
  • Kristen Hawthorne
    Howerton Eye Clinic, Austin, Texas, United States
  • Footnotes
    Commercial Relationships   Kaylin Arpaia, None; Kristen Hawthorne, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2021, Vol.62, 3458. doi:
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      Kaylin Arpaia, Kristen Hawthorne; A Unique Ophthalmic Presentation Leading to a Diagnostic Dilemma in Wegener’s Granulomatosis. Invest. Ophthalmol. Vis. Sci. 2021;62(8):3458.

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

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Purpose : A 22-year-old female with a biopsy proven history of Wegener’s granulomatosis (WG) maintained on prednisone 40mg daily, presented with a red, painful left eye of 3 weeks duration. She had been cared for in the prior 2 weeks by a referring ophthalmologist with reports of a slow growing, mass in the anterior chamber. Historical manifestations of her disease were sinus related as well as unilateral scleritis to the left eye. Her condition had been quiescent during her recent pregnancy which resulted in her second child three months prior. She had been on cyclophosphamide at the beginning of her diagnosis but after several years, she was taken off the alkylating agent and maintained on oral steroids alone.

Methods : On examination, her visual acuities were 20/70-1 right eye and LP left eye, with the majority of her vision in the left eye lost in 2018 to complications of anterior and posterior scleritis. Anterior segment examination of the left eye showed conjunctival hyperemia localized inferotemporally and a soft, ameboid, white mass in the anterior chamber measuring 7.5 x 5.5mm without corneal involvement. Concern for infectious or fungal etiology was high considering the patient was maintained chronically with 40mg prednisone daily. External photography of the white anterior segment mass was taken. (Figure 1, Figure 2) A preliminary diagnosis of infectious uveitis was made, and the patient was prescribed topical antibiotic and antifungal therapies with a plan for a biopsy/culture of the lesion with concurrent intracameral and subconjunctival antibiotic, antifungal and steroid therapy.

Results : Biopsy results showed that the mass was made entirely of inflammatory material, linking it to WG as opposed to the suspected infectious etiology.

Conclusions : This case shows a unique ophthalmic manifestation of WG in the anterior segment. While there are numerous reports detailing cases of episcleritis, anterior scleritis, ulcerative keratitis, and other inflammatory conditions of the eye associated with WG, to our knowledge there are no such reports of anterior segment masses. This case is interesting in that the inflammatory disease flared with the documented finding while on a high dose of oral steroids, leading to a diagnostic challenge. A review of current treatment recommendations and ophthalmic manifestations of WG accompany this case report as a guide for a challenging ophthalmic and systemic disease.

This is a 2021 ARVO Annual Meeting abstract.




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