Vasculitis

Retinal vasculitis from any cause may progress through the stages of inflammation, ischemia, neovascularization, and subsequent hemorrhagic and tractional complications. The early clinical manifestations are generally nonspecific, consisting of perivascular infiltrates and sheathing of the retinal vessels (vascular wall thickening with vessel involution; Fig 7-6). Involvement of exclusively retinal arteries or veins is uncommon. Veins tend to become inflamed earlier and more frequently than arterioles, and combinations are the rule. Retinal vasculitis may be caused by

Figure 7-6 Fundus photograph of retinal vasculitis in an eye of a patient with Crohn disease. Retinal hemorrhages and edema are present, as is prominent sheathing of the retinal vessels.

(Courtesy of Gary C. Brown, MD.)

• Behçet disease

• cat-scratch disease

• certain medications, such as rifabutin

• giant cell arteritis

• inflammatory bowel disease

• Lyme disease

• multiple sclerosis

• pars planitis

• polyarteritis nodosa

• sarcoidosis

• syphilis

• systemic lupus erythematosus

• toxoplasmosis

• viral retinitis

Masquerade syndromes should also be considered. See BCSC Section 9, Uveitis and Ocular Inflammation, for further discussion of most of these conditions and Section 5, Neuro-Ophthalmology, for discussion of multiple sclerosis and giant cell arteritis.

A primary occlusive retinal vasculopathy for which no cause can be found is termed Eales disease. This condition is an occlusive vasculopathy that usually involves the peripheral retina of both eyes and often results in extraretinal neovascularization with vitreous hemorrhage. It typically occurs in males, and there may be associated tuberculin hypersensitivity.

Susac syndrome is characterized by multiple branch retinal arterial occlusions and can be associated with hearing loss and, in rare cases, with strokes. It is most commonly diagnosed in women in their third decade, and there is no known cause. Susac syndrome is treated with corticosteroids or immunosuppression.

Chronic embolism or thrombosis without inflammation may result in a clinical picture that is indistinguishable from past retinal vasculitis. Evaluation includes a search for possible causes: cardiac valvular disease, cardiac arrhythmias, ulcerated atheromatous disease of the carotid vessels, and hemoglobinopathies.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) describes a syndrome characterized by the presence of retinal vasculitis, multiple macroaneurysms, neuroretinitis, and peripheral capillary nonperfusion. Systemic investigations are generally noncontributory, and oral prednisone has demonstrated little benefit. Capillary nonperfusion is often sufficiently severe to warrant panretinal photocoagulation (PRP).

Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.