Therapy Insight: The Recognition and Treatment of Retinal Manifestations of Systemic Vasculitis

Petros Aristodemou; Miles Stanford


Nat Clin Pract Rheumatol. 2006;2(8):443-451. 

In This Article

Retinal Manifestations of the Complications of Vasculitides or Their Treatment

Hypertensive Retinopathy and Choroidopathy

Systemic hypertension is a common complication of systemic vasculitis. Signs of hypertensive retinopathy and choroidopathy will vary, depending on the mode of onset and the severity of hypertension.[31] Severe, hypertensive retinopathy is characterized by flame hemorrhages and vascular leakage. This retinopathy results in the formation of retinal edema and hard exudates. Optic nerve swelling signifies a hypertensive crisis, and exudate can form a macular star around the fovea (Figure 6).

Figure 6.

Retinopathy in hypertensive crisis. A color fundus photograph that shows optic disk swelling, cotton-wool spots (blue arrow), hemorrhages (white arrow), retinal exudation and a macular star (green arrow).

Hypertensive choroidopathy is rare, and usually occurs in the setting of a hypertensive crisis. Acute, exudative retinal detachment can result from exudation from the choroid through the retinal pigment epithelium.[32] Chronic changes due to focal choroidal infarcts are recognized as small, dark spots surrounded by pale haloes, whereas choroidal vessels undergoing fibrinoid necrosis are visible as linear lesions.

Retinal Effects of Immunosuppression

The immunosuppressive effect of systemic drugs such as cyclophosphamide, azathioprine and corticosteroids can increase the risk of ocular infections such as toxoplasmosis, herpes- virus-related retinitis and tuberculosis. These infections can cause permanent vision loss in the absence of prompt treatment. Herpes simplex virus and varicella-zoster virus can cause a necrotizing retinitis, which starts in the fundal periphery and spreads to the center. Necrotizing retinitis is characterized by contiguous areas of white retinal necrosis, hemorrhages, and vasculitis of retinal arterioles. This clinical picture is termed acute retinal necrosis, and it can occur in both immunocompetent and immunocompromized hosts. High-dose intravenous antiviral therapy is often effective in arresting the progression of acute retinal necrosis, and in causing remission.

Cytomegalovirus typically causes a retinitis that starts in the posterior pole and involves the macula and the optic nerve early in its course (Figure 7).[33,34] Cytomegalovirus retinitis is treated with systemic ganciclovir or foscarnet.

Figure 7.

Cytomegalovirus retinitis. A color fundus photograph that shows contiguous areas of white retinal necrosis (blue arrow), vascular sheathing (green arrow), and hemorrhage (white arrow) in the retinal periphery.

The clinical appearance of the retinitis is often not discriminatory, and a vitreous-humor biopsy is essential, so that polymerase chain reaction assays for viral sequences can differentiate between the various types of viral retinitis and appropriate treatment can be chosen. Indeed, toxoplasmosis of the eye, as well as syphilitic and tuberculous chorioretinitis can all mimic the clinical picture of viral retinitis, and these etiologies should be borne in mind in atypical cases, or when the polymerase chain reaction assay for viral sequences in the vitreous humor is negative. Symptoms of floaters or reduced vision, particularly in a red eye, or the detection of retinal signs by the physician, should prompt an urgent ophthalmic referral.

Optic neuropathy is a well-described adverse event caused by methotrexate-induced folate deficiency. Patients might complain of a reduction in their visual acuity. The optic nerve can be swollen and/or pale. Formal evaluation of visual fields often reveals centrocecal defects, which extend from the blind spot to involve the central point of fixation.[35] Folate replacement might reverse this visual loss. As with all patients on long-term immunosuppression, there is an increased risk of malignancy, which might present in the eye as ocular lymphoma. It is beyond the scope of this article to discuss chloroquine-related and hydroxychloroquine-related retinal toxicity, which have been reviewed elsewhere.[36,37]


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