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

Summary and Introduction


A variety of retinal signs can occur in patients who have systemic vasculitides, or who experience complications of these diseases or their treatment. Although treatment of these retinal manifestations is usually the treatment of the systemic disease, specific treatment is occasionally indicated to preserve vision. The more prevalent of the systemic vasculitides are giant cell arteritis, polyarteritis nodosa, Wegener's granulomatosis, Churg–Strauss syndrome, relapsing polychondritis and systemic lupus erythematosus. Less frequently occurring vasculitides include Takayasu's arteritis, Goodpasture's disease, microscopic polyangiitis and Henoch–Schönlein purpura, as well as vasculitis secondary to scleroderma and rheumatoid arthritis. This article describes the pathogenesis, clinical features and treatment of retinal manifestations of systemic vasculitides.


Pathologically, the systemic vasculitides comprise a heterogenous group of disorders that are characterized by inflammatory cell infiltration and necrosis of blood vessel walls. This pathology can involve the blood supply to the eye, and cause retinal signs. The types of clinical signs seen in the posterior segment of the eye that relate to the various vasculitides are influenced by the diameter of vessel involved.[1] These signs range from microinfarcts (cotton-wool spots, as seen in systemic lupus erythematosus) to ischemia of the whole eye (in giant cell arteritis and Takayasu's arteritis). In the presence of scleral or orbital inflammation, other retinal signs can be evident. This article describes the pathogenesis of retinal signs that are associated with systemic vasculitis, and discusses their clinical features and treatment.

The Blood Supply to the Posterior Segment of the Eye. The posterior segment of the eye has two discrete circulations. The inner retinal layers are supplied by branches of the central retinal artery whereas the posterior ciliary arteries supply the choroid, which nourishes the outer retinal layers. Branches of the posterior ciliary arteries also supply the optic nerve head. Both the central retinal artery and the posterior ciliary arteries are, ultimately, branches of the ophthalmic artery (Figure 1).

Figure 1.

A schematic representation of the posterior retinal circulation. Parts of the blood supply to the optic disk, and the segmentation of the choroidal circulation are illustrated. The short posterior ciliary arteries (a to i) supply segments of the choroid (A to I) posterior to the equator of the globe.


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