When systemic lupus erythematosus affects vision: a rare presentation of this condition

Abstract

A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy. Steroid therapy alone was highly effective and was also accompanied by a normalisation of haemoglobin and lymphocyte counts, after which azathioprine was added. Hydroxychloroquine was introduced after resolution of retinal changes. Immunosuppressive therapy was progressively tapered over the course of 12 months and then discontinued, and the patient remains in remission 48 months after the initial presentation. Our patient exemplifies a very rare manifestation of systemic lupus erythematosus. We emphasise the importance of its early detection and complexity of treatment in order to reduce visual morbidity.

Keywords: immunology; retina; systemic lupus erythematosus.

Figure 1

(A) Hospital admission retinography, angiography and optical coherence tomography (OCT) showing cotton wool exudates (arrow), retinal vascular tortuosity; late leakage of macular vessels (arrowhead) and macular oedema (asterisk), respectively; (B) week 4 retinography showing improvement of cotton-wool spots (arrow) and a marked decrease in vascular tortuosity. OCT documented a macular oedema improvement (asterisk); (C) week 9 retinography, and OCT showing rare retinal exudates (arrow), important decrease in vascular tortuosity and restoration of normal macula structure (asterisk).

Figure 2

Sequential therapy and disease activity markers throughout follow-up. The patient remains in clinical remission with laboratory quiescence after a 48-month follow-up. dsDNA, double-stranded DNA; HCQ, hydroxychloroquine.