Diagnostic dilemma in an occlusive vasculitis case – infective, immune-mediated or masquerade?
Purpose
To describe a case of left occlusive retinal vasculitis with severe ischemia in a patient with possible IgG-4 related orbital disease. Confounders to etiology include positive Cytomegalovirus (CMV) PCR from the aqueous humor and history of complete internal carotid artery occlusion ipsilaterally. A review of the literature was done to discuss this rare case.
Methods
A chart review of the patient from August 2019 to May 2022 and a literature review of CMV, IgG-4 ophthalmic disease and ocular ischemic syndrome as possible etiologies was performed.
Results
A 65-year-old Chinese male was referred for 3 days of left blurring of vision. He has been followed-up by Rheumatology and the Oculoplastics service for possible IgG-4 related disease with recurrent dacryoadenitis. Clinical examination revealed mild anterior segment inflammation, significant vascular sheathing, neovascularization at the disc, and a twig retinal artery occlusion. Fluorescein angiography showed subclinical inflammation in the right eye and widespread capillary fallout in the left eye. Extensive etiological workup revealed positive CMV PCR from the aqueous and persistence of high IgG-4 titer. The patient received intravenous methylprednisolone and steroid-sparing immunomodulatory therapy, and intravitreal ranibizumab with panretinal photocoagulation. Clinical course was complicated by CMV retinitis treated with valganciclovir successfully. Literature review showed there is no similar precedence.
Conclusion
This is the first reported case of occlusive retinal vasculitis in possible IgG-4 disease complicated with CMV retinitis. A successful outcome will depend on multiple modalities of treatment titrated to individual patient’s condition.
Conflict of interest
No
Authors 1
Last name
THNG
Initials of first name(s)
ZX
Department
Ophthalmology
City
Singapore
Country
Singapore
Authors 2
Last name
TEOH
Initials of first name(s)
SCB
Department
Ophthalmology
City
Singapore
Country
Singapore
Authors 3
Last name
Nguyen
Initials of first name(s)
QD
Department
Ophthalmology
City
Palo Alto
Country
United States
This website uses cookies to ensure you get the best experience on our website.
Learn more