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Therapy Insight: the recognition and treatment of retinal manifestations of systemic vasculitis

Nature Clinical Practice Rheumatology volume 2, pages 443–451 (2006)Cite this article

Abstract

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.

Key Points

  • The pathology of the systemic vasculitides can involve the blood supply to the eye, causing retinal signs

  • The types of posterior segment clinical signs relating to the various vasculitides are influenced by the diameter of vessel involved

  • With the exception of giant cell arteritis and hypertensive retinopathy, fundal signs related to systemic vasculitides are uncommon or even rare

  • The vasculitic process typically affects the retinal arteries rather than the veins, and these signs are not specific to any type of vasculitis

  • Often, the development of retinal signs heralds a relapse of the underlying vasculitis, and urgent referral to a rheumatologist is required

  • The possibility that retinal signs might be secondary to iatrogenic immunosuppression should be borne in mind, as rapid and specific treatment for retinal infections might preserve vision

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Figure 1: A schematic representation of the posterior retinal circulation.
Figure 2: Systemic lupus erythematosus retinopathy.
Figure 3: Giant cell arteritis.
Figure 4: Fluorescein angiograms showing normal choroidal perfusion, and delayed choroidal perfusion in giant cell arteritis.
Figure 5: Posterior scleritis.
Figure 6: Retinopathy in hypertensive crisis.
Figure 7: Cytomegalovirus retinitis.

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Author information

Authors and Affiliations

  1. King's College London,

    Miles Stanford

  2. Medical Eye Unit at St Thomas' Hospital, London, UK

    Miles Stanford

  3. Specialist Registrar in Ophthalmology at the Royal United Hospital, Bath, UK

    Petros Aristodemou

Authors
  1. Petros Aristodemou
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  2. Miles Stanford
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Corresponding author

Correspondence to Miles Stanford.

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The authors declare no competing financial interests.

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Aristodemou, P., Stanford, M. Therapy Insight: the recognition and treatment of retinal manifestations of systemic vasculitis. Nat Rev Rheumatol 2, 443–451 (2006). https://doi.org/10.1038/ncprheum0268

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