Reversible posterior leukoencephalopathy in a patient with systemic sclerosis/systemic lupus erythematosus overlap syndrome

A 39-year-old woman with systemic sclerosis/systemic lupus erythematosus overlap syndrome was admitted with fever. This followed a second course of pulsed intravenous cyclophosphamide for relapse of lupus nephritis WHO class IV with crescents. Her past history included Raynaud’s phenomenon, pulmonary fibrosis, oesophageal dysmotility and membranous glomerulonephritis causing nephrotic syndrome 8 years previously. She had also had seizures in pregnancy associated with pre-eclamptic toxaemia. During the admission, her blood pressure rose to 170/100mmHg. She developed cortical blindness followed by status epilepticus. A head computed tomography (CT) scan showed bilateral subcortical white matter changes in the posterior lobes, suggestive of posterior leukoencephalopathy. A magnetic resonance imaging (MRI) scan showed high T2 signal in the left cerebellar white matter and both occipital poles involving grey and white matter (Figure 1). A magnetic resonance venogram (MRV) was normal (Figure 2). Her blood pressure was controlled with intravenous nitrates and prostacyclin. Her symptoms resolved rapidly. A repeat MRI scan 2 weeks later showed resolution of some of the changes (Figure 3). A repeat renal biopsy showed crescent formation in two glomeruli with no evidence of malignant hypertension. Three weeks later, her blood pressure rose once again to a maximum of 150/90mmHg. She re-developed the cortical blindness, generalized hypertonicity and grand mal seizures. A thirdMRI scan showed that the findings of posterior leukoencephalopathy had mostly resolved, but there were now new bright subcortical white matter changes in the right preand postcentral gyri, both middle frontal gyri and the left superior frontal gyrus, suggesting the possibility of an acute vasculitis (Figure 4). Once again the MRV was normal. Aggressive blood pressure control and immunosuppression with mycophenolate mofetil led to a resolution of all neurological symptoms and signs, and recovery of renal function over the ensuing 8 weeks. She remains well with immaculate blood pressure control and maintenance mycophenolate mofetil as immunosuppression.