Dramatic effect of bolus cyclophosphamide in a severe case of lupus retinopathy

Abstract

Purpose To report the dramatic effect of bolus cyclophosphamide in a severe case of lupus retinopathy. Methods A 31‐year‐old woman with active systemic lupus erythematosus (SLE) consulted for asymmetric rapidly progressive decreased visual acuity (8/10 P2 OD, 1/20 P14 OS). Fundus examination disclosed bilateral macular oedema, numerous peripapillary cotton wool spots and hemorrhages suggestive of the diagnosis of lupus retinopathy. Clinical and biological criteria allowed the diagnosis of severe multi systemic SLE with lupus glomerulonephritis. Treatment based on oral 1mg/kg daily steroids and 6 intravenous cyclophosphamide cures (100mg/m2). Rapid improvement in systemic and biological signs of SLE was observed with parallel disappearance of retinal signs. Full recovery of visual acuity was obtained within 6 months. Results Prevalence of SLE varies between 10 and 50 p 100,000. Presence of ophthalmological manifestations is estimated between 5 and 50% of the patients depending on the series, and the most frequent one is lupus retinopathy. First line treatment is usually based on oral corticosteroids. Immunosuppressive drugs may be used as part of a steroid‐sparing strategy or for resistant forms of the disease. In case of severe acute cases, as in our patient, intensive treatment combining steroids and cyclophophamide cures enables regression of retinal signs and improvement of visual acuity, along with clinical and self‐immune and inflammatory tests. Conclusion In severe forms of SLE a treatment combining cyclophosphamide cures and steroids is mandatory to obtain not only improvement of systemic manifestations but also complete visual recovery.