Retinal Vasculitis – December 2016

December 2016

Case Presentation

A 19 year old was referred to Illinois Retina Associates for light sensitivity, blurry vision, and floaters in her left eye.

She was known for Sjogren’s syndrome, anemia and migraines, and was taking Plaquenil for the past year.

She had a history of intermittent floaters OS since an episode of retinal vasculitis OS which occurred shortly after an episode of mononucleosis 2 years ago. The causative agent for the mononucleosis was believed to be the Epstein-Barr virus (EBV), confirmed through laboratory testing.

Examination

Her corrected visual acuity was 20/20 in the right eye, and 20/30 in the left eye, not improved with pinhole. Anterior segment examination was unremarkable. On posterior segment examination, there was a mild vitreous hemorrhage, scattered areas of vascular sheathing and neovascularization OS. The exam of the right eye was within normal limits.

Diagnostic Testing

Fundus photography (Figure 1) and fluorescein angiogram (Figures 2 & 3) confirmed the presence of vitreous hemorrhage, areas of retinal ischemia and neovascularization OS. Optical coherence tomography showed irregularity of retinal layers but no edema OS (Figure 4). All imaging studies were normal OD.

Figure1
Figure 1
Figure1
Figure 2
Figure1
Figure 3
Figure1
Figure 4

Differential Diagnosis

  • Infectious Vasculitis
    • Viral (CMV, VZV, EBV)
    • Bacterial (Syphilis, Tuburculosis)
  • Inflammatory Vasculitis
    • Sarcoidosis
    • Behcet Disease
    • Systemic Lupus Erythematosis
    • Inflammatory Bowel Disease
    • Multiple Sclerosis
  • Idiopathic Retinitis, vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome

Diagnosis

All laboratory investigations were normal and the diagnosis was established as EBV-related retinal vasculitis with secondary retinal ischemia, neovascularization and vitreous hemorrhage.

Follow-Up

Given the presence of extensive retinal ischemia, multiple areas of neovascularization and vitreous hemorrhage, pan-retinal photocoagulation treatment was recommended to decrease risk of recurrent hemorrhage or other complications such as retinal traction or detachment. The vitreous hemorrhage spontaneously improved over time.

Discussion

Many local as well as systemic diseases may lead to retinal vasculitis. It is crucial to identify the cause of the vasculitis and rule out systemic involvement to prevent complications. Some forms of retinal vasculitis may benefit from systemic immunomodulatory therapy.

If the vasculitis is only affecting the retina, fluorescein angiogram is essential in order to identify retinal ischemia and neovascularization. In such advanced cases, pan-retinal photocoagulation can be applied to prevent visual loss from vitreous hemorrhage or retinal detachment.

Macular edema can also be present in vasculitis. Macular OCTs are helpful in identifying and quantifying the edema. When present, macular edema may result in decreased vision and may respond to drops (Corticosteroids, non-steroidal anti-inflammatories), local steroid injections, or systemic immunosuppressants.

References

Keorochana N. A Case Report of Epstein-Barr Virus-associated Retinal Vasculitis: Successful treatment using only acyclovir therapy. Int. Med Case Rep J. 2016 Jul 25; 9:213-8.

Rosenbaum JT, Sibley CH, Lin P. Retinal Vasculitis. Curr Opin Rheumatol 2016 May; 28(3):228-35.

Talat L, Lightman S, Tomkins-Netzer O. Ischemic Retinal Vasculitis and its management. J. Ophthalmol 2014;2014:197675