Abstract
BackgroundPosterior reversible encephalopathy syndrome (PRES) has been increasingly identified in patients with systemic lupus erythematosus (SLE) owing to the advance in neuroimaging techniques. Prompt diagnosis is pivotal to improve its outcome. To analyze the clinical and radiographic profile of PRES in patients with SLE and search for the appropriate treatment strategy PRES in SLE.MethodsSLE patients who fulfilled the diagnostic criteria for PRES from August 2008 to January 2011 were evaluated at baseline, and followed to determine clinical outcomes. Data were analysis on clinical characteristics, laboratory abnormalities, treatment details, and outcomes.ResultsTen episodes of PRES in patients with SLE were identified. All patients were female, mean age of onset was 22.93 ± 2.48 years, and SLEDAI at the onset of PRES were 25.8 ± 5.7. All cases had acute onset of headache, altered mental status, stupor, vomiting, cortical blindness and seizures. Neurological symptoms were the initial manifestation of SLE in three cases. Head magnetic resonance imaging (MRI) demonstrated posterior white matter edema involving the parietal, temporal and occipital lobes, which were more conspicuous on T2 weighted spin echo and diffusion-weighted MR imaging (DWI) than on computed tomography (CT) scan. Complete clinical and radiographic recovery was observed in 8 patients after prompt treatment with corticosteroids.ConclusionsPRES might be due to lupus per se besides other traditional causative factors such as hypertension. PRES might be an underestimated variant of “reversible neurological deficits” in SLE. Prompt recognition and timely management is important to prevent permanent neurological deficits.
Highlights
Posterior reversible encephalopathy syndrome (PRES) has been increasingly identified in patients with systemic lupus erythematosus (SLE) owing to the advance in neuroimaging techniques
Posterior reversible encephalopathy syndrome (PRES) was first reported by Aisen et al in 1985 [7], which was a clinical entity characterized by headache, nausea, vomiting, seizures, conscious disturbance and visual disorder with predominantly white matter abnormalities of the parieto-occipital lobes on neuroimaging [8,9]
Literature review showed that severe hypertension (> 170/110 mmHg) and renal failure were present in the majority of previously reported cases of SLE with PRES, whereas SLE patients might develop reversible focal deficits that respond to steroid therapy [13]
Summary
Posterior reversible encephalopathy syndrome (PRES) has been increasingly identified in patients with systemic lupus erythematosus (SLE) owing to the advance in neuroimaging techniques. Posterior reversible encephalopathy syndrome (PRES) was first reported by Aisen et al in 1985 [7], which was a clinical entity characterized by headache, nausea, vomiting, seizures, conscious disturbance and visual disorder with predominantly white matter abnormalities of the parieto-occipital lobes on neuroimaging [8,9]. Literature review showed that severe hypertension (> 170/110 mmHg) and renal failure were present in the majority of previously reported cases of SLE with PRES, whereas SLE patients might develop reversible focal deficits that respond to steroid therapy [13]. The peculiar role of SLE itself in the occurrence of PRES was not clear since PRES could be a manifestation of lupus disease activity or a consequence of immunomodulatory therapy, making the diagnosis and treatment challenging. It was important to recognize the condition early in order to minimize potential for irreversible central nervous system damage
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