Combined central retinal artery and vein occlusion as the presenting manifestation of systemic lupus erythematosus

A 14-year-old girl visited to our outpatient department complaining of sudden loss of vision in her left eye (LE) for one day. Visual acuity in the right eye (RE) was 20/20 and hand motion in LE. LE had relative afferent pupillary defect. LE fundus revealed combined central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO) [Fig. 1]. Anterior and posterior segment findings were unremarkable in RE. Despite aggressive treatment, there was no improvement in the visual acuity of LE. Next day, she developed characteristic erythematous butterfly rash and multiple raised patchy rashes over the head, trunk and arms. Laboratory investigations revealed increased erythrocyte sedimentation rate, elevated levels of C-reactive protein, antinuclear antibody, double-stranded DNA and slightly decreased complement-3 and complement-4. Lupus anticoagulant and anticardiolipin antibodies were negative. She developed pancytopenia and lupus nephritis. Diagnosis of systemic lupus erythematosus (SLE) was made. Despite vigorous treatment, patient did not respond and died of terminal cardio-respiratory arrest due to bilateral aspiration pneumonitis with type 1 respiratory failure.

Figure 1.

Left eye fundus image showing disc oedema, pale and oedematous retina, engorged and tortuous retinal veins, scattered intraretinal heamorrhages in all quadrants and a cherry-red macular spot, suggesting combined central retinal artery and vein occlusion

Combined CRAO and CRVO as the presenting manifestation of SLE without raised antiphospholipid antibody levels has been rarely reported.[1,2] This case reminds us that SLE must be considered in the rare instance in which the patient presents with combined occlusion.

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