Uveitis

Case 7

Patient Presentation: A 61-year-old female presented to a uveitis clinic for routine hydroxychloroquine screening. The patient’s past medical history was significant for systemic lupus erythematosus, for which she was treated with Plaquenil for the past 6-months. The patient was asymptomatic on presentation. Visual acuity was 20/20 OU, with IOPs measuring 12 OD and 13 OS. Slit lamp examination was within normal limits. Dilated fundus examination revealed retinal vascular sheathing OU with dot-blot hemorrhages OS > OD. On further questioning based on the retinal findings, the patient later disclosed a prior diagnosis of antiphospholipid antibody syndrome and previous transient ischemic attacks (TIAs), which were not noted in the referral letter. She was on warfarin but not on any immunomodulatory agent other than the Plaquenil. Optos widefield fundus photos are shown below:

This fundus photo of the right eye in particular shows retinal vascular sheathing and areas of neovascularization (superonasally).

2 IVFA images of the right eye were taken (roughly 14 months apart), demonstrating peripheral ischemic areas/vascular blunting with multiple pinpoint areas of leakage in the first photo progressing to neovascularization superonasally in the second photo.

An OCT Angiography (OCTA) was performed and shown below:

Question: What abnormalities are noted on the two OCTA images above?

Question: Based on the abnormalities on OCTA, what disease process could be most likely occurring?

Question: What is the most likely diagnosis?

Question: What would the next best step be in management of this condition?

References

Cunningham ET, Zierhut M. Retinal Vasculitis. Ocular Immunology and Inflammation. 2020 Nov 16;28(8):1159–62.

Ghembaza A, Saadoun D. Management of Antiphospholipid Syndrome. Biomedicines. 2020 Nov 17;8(11):508.

Learning Objectives:

1. Identify OCT features of occlusive retinal vasculitis due to systemic conditions such as antiphospholipid syndrome.

2. Understand basic management of systemic conditions that can cause occlusive retinal vasculitis and the role the ophthalmologist may play in co-managing systemic care.

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