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Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature

Department of Internal Medicine

J. Sellam

Department of Internal Medicine

N. Costedoat-Chalumeau

Department of Internal Medicine, nathalie.costedoat{at}psl.aphp.fr

D. Le Thi Huong

Department of Internal Medicine

A. Combes

Department of Intensive Care Unit

N. Tieulié

Department of Internal Medicine

J. Haroche

Department of Internal Medicine

Z. Amoura

Department of Internal Medicine

A. Nieszkowska

Department of Intensive Care Unit

J. Chastre

Department of Intensive Care Unit

D. Dormont

Department of Neuroradiology, Centre Hospitalier Universitaire Pitié-Salpêtrière, 83 Boulevard de l'Hôpital, 75013 Paris, France

J-C. Piette

Department of Internal Medicine

Posterior reversible encephalopathy syndrome (PRES) associates various neurological manifestations (headaches, seizures, altered mental status, cortical blindness, focal neurological deficits, vomiting) and transient changes on neuroimaging consistent with cerebral edema. Posterior reversible encephalopathy syndrome mainly occurs in the setting of hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs.

We report four cases of PRES complicating systemic lupus erythematosus (SLE). In all our cases, renal involvement and hypertension were present. Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases, but one who had only partial regression related to cerebral hemorrhage.

Including our cases, we reviewed a total of 46 patients with SLE and PRES. Their clinical and radiological presentation was not specific. The peculiar role of SLE itself in the occurrence of PRES was not clear, since hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%) were often present. The hypertension and other worsening factors should be treated. Finally, the evolution of this clinical and radiological spectacular syndrome is generally rapidly favorable. Lupus (2008) 17, 139—147.

Key Words: anti-phospholipid syndrome • hypertensive encephalopathy • posterior reversible encephalopathy syndrome • systemic lupus erythematosus

Lupus, Vol. 17, No. 2, 139-147 (2008)
DOI: 10.1177/0961203307085405


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