Posterior reversible encephalopathy syndrome: an emerging disease manifestation in systemic lupus erythematosus

Abstract

Objectives: Posterior reversible encephalopathy syndrome (PRES) is a rare feature of systemic lupus erythematosus (SLE) that can present with seizures, visual disturbance, and/or hypertension. We describe 7 new cases of PRES in SLE patients that highlight some of the diagnostic and treatment dilemmas of this condition.

Methods: We obtained informed consent from patients for the review of their cases. A comprehensive literature search was done to find all published cases of PRES in SLE and these were compared with our cohort.

Results: Including our series, there are 66 cases of PRES in SLE patients published in the English literature to date. Our series had 2 main distinguishing features when compared with the available literature: only 43% of our patients presented with seizures compared with 83 to 95% in the reported literature and over 50% of our cases of PRES occurred in newly diagnosed SLE.

Conclusions: PRES is a more frequent feature of SLE than previously thought and may be 1 of the presenting manifestations of SLE. Whether PRES is a manifestation of SLE disease activity and of its treatment or whether it represents a neuropsychiatric manifestation of SLE remain to be determined. Consequently there continues to be controversy as to whether immunosuppression should be used in addition to supportive measures in the treatment of PRES in SLE.