Life threatening pericardial and neurologic presentations in systemic lupus erythematosus

Abstract

Background: Posterior reversible encephalopathy syndrome (PRES) is characterized by altered mental status, visual perception abnormalities and seizures. Systemic lupus erythematosus (SLE) is a rare etiology of PRES and affects less than 1% of SLE patients. We present a unique case of a new-onset SLE patient presenting with borderline cardiac tamponade requiring emergent pericardiocentesis and hospital course complicated by intractable seizures found to have PRES on imaging.

Case presentation: A 21-year-old female underwent outpatient evaluation for new-onset SLE presenting to the emergency room with dyspnea. She was found to have a large circumferential pericardial effusion with borderline tamponade features requiring emergent pericardiocentesis. On her fifth hospital day, she developed generalized motor seizure (tonic-clonic) with magnetic resonance imaging (MRI) revealing bilateral parieto-occipital edema consistent with a radiographic diagnosis of PRES. Induction therapy with mycophenolate was started and follow-up studies showed improvement in the cortical and subcortical regions on MRI with no evidence of recurrent pericardial effusion.

Conclusions: PRES is a rare complication of lupus and has been infrequently described in literature. It is thought that the association is secondary to the effect of SLE on endothelial dysfunction and the effect of cytotoxic medication on the loss of autoregulation of systemic hypertension. It is important to recognize this condition and its significant associations as early detection and intervention often lead to favorable outcomes. To our knowledge, this is the first case report of SLE with associations of both PRES and circumferential pericardial effusion with tamponade physiology, both of which are rare in the SLE patient population.