Childhood-onset Systemic Lupus Erythematosus Complicated by Posterior Reversible Encephalopathy Syndrome in The Context of Kidney Dialysis and Plasma Exchange in Pediatric Lupus

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic condition characterized by reversible vasogenic edema on neuroimaging. Its various manifestations include altered mental status, headache, visual changes, and seizure activity along with a primarily posterior leukoencephalopathy on imaging studies. PRES has been found in the setting of eclampsia, hypertension, uremia, malignancy, transplantation, autoimmune disease, and/or the use of immunosuppressive drugs (specifically cyclosporine). Recently, it has been described more frequently in children with systemic lupus erythematosus (SLE) and it is often difficult to distinguish from central nervous system lupus. In this paper, we describe five cases of pediatric SLE complicated by PRES. Three of the patients were receiving dialysis, one was receiving plasmapheresis. None of the patients were on cyclosporine. In our center, we follow over 60 children with SLE and these are the only five cases diagnosed with PRES as part of their disease course. Many of our patients have associated lupus nephritis and hypertension and are on multiple immunosuppressive medications. We hypothesize that it is the fluid shifts and rapid increase in blood pressure during these treatments that contributed to the development of PRES in these patients.