Juvenile systemic lupus erythematosus presenting as retinal vasculitis.

Abstract

A 15-year-old boy presented with a 2-week history of decreased vision in his right eye and a painful rash over his toes. On examination he had pallor, a vasculitic rash over the toes of both feet, and a visual acuity of 6/24 in the right eye. Fundoscopy showed vascular sheathing of the central retinal vessels, cotton wool spots, and dot haemorrhages, consistent with retinal vasculitis (figure A). Blood tests showed a haemoglobin concentration of 8 g/dL, white cell count of 1700 per μL (normal range 4000–11 000 per μL), and platelet count of 70 000 per mL (normal range 150 000–400 000 per mL). His renal and hepatic parameters were normal. Tests for antinuclear antibodies and anti-double-stranded DNA antibodies were positive, and direct Coomb's test was positive. Antinuclear antibody blot test showed positivity for anti-Sjögren's-syndrome-related antigen A antibodies and anti-double-stranded DNA antibodies. Complement concentrations were normal and tests for antiphospholipid antibodies were negative.