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Case Reports
. 2022 May 5;6(6):467-469.
doi: 10.1177/24741264221076358. eCollection 2022 Nov-Dec.

Granulomatosis With Polyangiitis Presenting as Occlusive Retinal Vasculitis

Madeline L D'Aquila  1 , Vishak J John  1   2
Affiliations
Case Reports

Granulomatosis With Polyangiitis Presenting as Occlusive Retinal Vasculitis

Madeline L D'Aquila et al. J Vitreoretin Dis. .

Abstract

Purpose: We discuss an unusual case of granulomatosis with polyangiitis (GPA) presenting as anterior uveitis with occlusive retinal vasculitis.

Methods: A case report is presented.

Results: A 60-year-old woman with a history of autoimmune disease presented to the retina clinic with red eyes and blurry vision in both eyes. An examination showed anterior uveitis with retinal vasculitis, and topical steroids were started in both eyes. One month later, the patient's vision worsened and an optical coherence tomography scan showed new central cystoid macular edema in the left eye. An antivascular endothelial growth factor injection was given. The next day, her vision was "black" in the left eye and a fundus examination showed global ischemia. A comprehensive uveitis workup was positive for cytoplasmic-staining antineutrophilic cytoplasmic antibody. A diagnosis of GPA was confirmed with a renal biopsy.

Conclusions: Physician awareness of ocular GPA presentation is vital, and GPA management is most successful with a multidisciplinary team.

Keywords: OCT; autoimmune disease; fundus autofluorescence granulomatosis with polyangiitis; macular edema; retina; retinal vascular disease; uveitis.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Late-phase fluorescein angiograms of the (A) right eye and (B) left eye show arteriovenous fistulas and optic nerve leakage, respectively, and temporal vascular remodeling bilaterally.
Figure 2.
Figure 2.
Optical coherence tomography scan of the left eye shows new central cystoid macular edema.
Figure 3.
Figure 3.
(A) Fundus photograph of the patient’s left eye shows hemorrhages globally and optic nerve swelling. (B) Fundus autofluorescence highlights hemorrhages in the left eye.
Figure 4.
Figure 4.
Renal biopsy with a methenamine silver-periodic acid–Schiff stain at 400× magnification shows affected and unaffected glomeruli. The glomerulus on the lower right is involved by a large cellular crescent. The glomerulus without crescents (left) is normocellular. Given the negative immunofluorescence, the constellation of findings is consistent with pauci-immune crescentic glomerulonephritis, which is typical of renal involvement by granulomatosis with polyangiitis.
See this image and copyright information in PMC

References

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