Transient blindness and seizures in severe lupus nephritis.

Abstract

A 19-year-old woman with severe lupus nephritis was brought to the emergency department for blindness and generalized seizures. Class IV lupus nephritis had been diagnosed 1 year earlier and treated with corticosteroids and mycophenolate mofetil. Three months before admission, serum creatinine was 1.0 mg/dL. Poor compliance to prescribed therapy 2 months before admission led to worsening nephrotic syndrome (proteinuria 22 g/day) and renal failure (creatinine 5.1 mg/dL), with a systemic lupus erythematosus (SLE) disease activity index of 39. A second kidney biopsy confirmed severe class IV lupus nephritis with crescents in ∼50% of the glomeruli and a histological activity index of 18/42. Despite aggressive immunosuppressive therapy including corticosteroids, cyclophosphamide and rituximab, haemodialysis had to be initiated 2 weeks before the current admission. On the morning of admission, she complained of acute onset blindness and headache, and developed generalized tonic–clonic seizures. Physical examination revealed hypertension (155/100 mmHg), obtundation and visual loss. There were no hydroelectrolytic disturbances, and cerebrospinal fluid examination was normal. Fundoscopic examination showed a grade 2 hypertensive retinopathy. Magnetic resonance imaging showed bilateral parietooccipital hyperintensities on fluid-attenuated inversion recovery (FLAIR) sequences (Figure 1A), consistent with cerebral oedema.