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 Findings in Small-vessel Vasculitis

Wegener's Granulomatosis

Retinal complications of Wegener's granulomatosis are rare, and include retinal artery occlusions, cotton-wool spots and choroidal infarcts.[15] Posterior scleritis can cause choroidal folds (Figure 5), exudative retinal detachment and compressive optic neuropathy.[8] An anterior ischemic optic neuropathy similar to that seen in giant cell arteritis has been reported.[16] The presence of retinal signs usually signifies a major relapse in the underlying disease, and this relapse needs to be treated aggressively.

Churg–Strauss Syndrome

Retinopathy in Churg–Strauss syndrome has been described in a few reports.[17] This retinopathy consists of an ischemic vasculitis that gives rise to AION associated with retinal arterial or venous occlusions, and typically occurs in patients positive for serum perinuclear antineutrophil cytoplasmic antibodies (pANCA). It has been advocated that patients who are pANCA-positive should be considered for prophylactic steroid therapy, given that once this retinopathy develops, visual prognosis is poor despite steroid treatment.[17] The value of this approach has not been formally tested. Nevertheless, posterior-segment complications related to ischemic vasculitis can develop even in the absence of positive pANCA titers, and these can be equally visually devastating.[18]

Relapsing Polychondritis

Scleritis is the most common ocular manifestation of relapsing polychondritis; in one study, this sign was present in 51 out of 117 patients, and was the presenting feature in 6 patients.[19] Posterior scleritis can result in choroidal folds, exudative retinal detachment, and optic nerve compression.

In the aforementioned series, nine patients had retinopathy consisting of microaneurysms, hemorrhages and cotton-wool spots. Two other patients had retinal vasculitis associated with vein occlusions.[19] AION can also occur in relapsing polychondritis.[20] Where present, scleritis will need high-dose immunosuppression to control pain and restore vision. Ischemic complications require attention to the management of the underlying disease.

Systemic Lupus Erythematosus (SLE)

Retinopathy is the second most common ophthalmic manifestation of SLE (after secondary Sjögren syndrome), with reported incidences ranging from 3% in the outpatient setting[21] to 29% in admitted patients.[22] SLE retinopathy is characterized by the presence of cotton-wool spots (Figure 2). Retinal hemorrhages and microaneurysms might also occur. Although SLE retinopathy has a good visual prognosis, it is associated with major relapses of systemic disease,[21] and its presence warrants an immediate referral for rheumatologic consultation. An occlusive retinopathy with poor visual prognosis has been described in patients with SLE. This complication is very rare and is associated with cerebral vasculitis and increased mortality.[23]

Choriodopathy is a rare finding in patients with SLE, and few cases have been reported in the literature.[24] Choroidopathy can be asymptomatic with no ophthalmologic signs,[25] or it can result in serous detachments of the retinal pigment epithelium or the retina.[26] Lupus choriodopathy indicates active disease and it is associated with cerebral vasculitis.[24] A variety of presentations of optic neuropathy has been reported in association with SLE, including cases of papilledema secondary to cerebral venous thrombosis.[27]

The pathogenesis of SLE retinopathy and choroidopathy seems to arise from fibrinoid necrosis and vessel occlusion secondary to immune-complex deposition.[28] Associated antiphospholipid syndrome is also a risk factor for occlusive disease.[29,30] The retinal manifestations of other systemic vasculitides are summarized in Table 2 .


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