Abstract

Ischemic retinal vasculitis is an inflammation of retinal blood vessels associated with vascular occlusion and subsequent retinal hypoperfusion. It can cause visual loss secondary to macular ischemia, macular edema, and neovascularization leading to vitreous hemorrhage, fibrovascular proliferation, and tractional retinal detachment. Ischemic retinal vasculitis can be idiopathic or secondary to systemic disease such as in Behçet's disease, sarcoidosis, tuberculosis, multiple sclerosis, and systemic lupus erythematosus. Corticosteroids with or without immunosuppressive medication are the mainstay treatment in retinal vasculitis together with laser photocoagulation of retinal ischemic areas. Intravitreal injections of bevacizumab are used to treat neovascularization secondary to systemic lupus erythematosus but should be timed with retinal laser photocoagulation to prevent further progression of retinal ischemia. Antitumor necrosis factor agents have shown promising results in controlling refractory retinal vasculitis excluding multiple sclerosis. Interferon has been useful to control inflammation and induce neovascular regression in retinal vasculitis secondary to Behçet's disease and multiple sclerosis. The long term effect of these management strategies in preventing the progression of retinal ischemia and preserving vision is not well understood and needs to be further studied.

Highlights

  • Retinal vasculitis is a sight-threatening inflammatory condition, occurring in approximately one in every eight eyes with uveitis [1]

  • The main concern with retinal vasculitis is the risk of developing vasooclusion and retinal ischemia that can lead to serious sight threatening manifestations

  • In a small retrospective study of 13 patients with multiple sclerosis (MS) related uveitis, ten of which were associated with retinal vasculitis, showed promising results with improvement of visual acuity in 71% of the eyes while a corticosteroid sparing effect was achieved in all cases [100]

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Summary

Background

Retinal vasculitis is a sight-threatening inflammatory condition, occurring in approximately one in every eight eyes with uveitis [1]. The main concern with retinal vasculitis is the risk of developing vasooclusion and retinal ischemia that can lead to serious sight threatening manifestations. Vascular changes in uveitis are characterized by perivascular infiltration of lymphocytes resulting in perivasculitis rather than a true vasculitis of the vessel wall [6, 7]. SLE: systemic lopus erythematosus; APHA: antiphospholipid antibody syndrome; IRVAN: idiopathic retinal vasculitis, arteriolar macroaneurysms, and neuroretinitis; CMV: cytomegalovirus; HIV: human immunodeficiency virus; HTLV-1: human T-cell lymphoma virus type 1; APMPPE: acute posterior multifocal placoid pigment epitheliopathy; GPA: granulomatosis with polyangiitis. (NV) at optic disc (NVD) or elsewhere in the retina (NVE) These fragile new vessels bleed resulting in vitreous hemorrhage (VH), fibrovascular proliferation, and subsequent tractional retinal detachment. The risk of visual loss in cases with retinal ischemia relates to involvement of posterior pole as in macular edema and macular ischemia or due to stimulating neovascularization

Presumed Tuberculous Retinal Vasculitis
Behçet’s Disease
Systemic Lupus Erythematosus
Antiphospholipid Syndrome
Sarcoidosis
Multiple Sclerosis
Other Causes of Occlusive Retinal Vasculitis
Treatment
Findings
10. Conclusion
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