Posterior reversible encephalopathy syndrome as a complication of acute lupus activity

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Abstract

Objectives

We aimed to describe the clinical and imaging characteristics; associated risk factors and neurological outcome of posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE).

Methods

From October 2001 to January 2007, we identified patients with SLE and the criteria for PRES in our institution, which is a tertiary-care referral center for patients with SLE; the patients were evaluated at baseline and followed to determine the clinical outcome.

Results

We identified 22 episodes of PRES in 21 patients; 20 (95.2%) were women, mean age of onset was 24.9 ± 8.6 years, all patients had high systemic activity (SLEDAI scores from 12 to 39). Acute hypertension was observed in 18 episodes (81.8%), and renal failure in 16 (72.7%); only 3 patients were on cyclophosphamide at the time of the onset of PRES. Persistent neurological deficit was observed in 2 cases; one patient died during the acute episode.

Conclusions

PRES is a central nervous system syndrome that is observed in SLE patients. It was associated mainly to high systemic activity, acute hypertension, and renal failure. Although reversibility is common, residual neurological damage may be observed.

Introduction

Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological syndrome first described by Hinchey et al. in 1996 [1]; its main pathogenic mechanism is a disruption of the blood–brain barrier (BBB) and secondary leakage of plasma into the brain parenchyma. It is clinically characterized by headache, decreased level of alertness, seizures and visual loss of cortical type. Multiple treatments and clinical conditions have been associated to this disorder, including: immunotherapy [2], erythropoietin treatment [3], organ transplantation [4], transfusions [5], chemotherapy [6], and Guillain-Barré syndrome [7].

Reports of patients with systemic lupus erythematosus (SLE) who developed PRES, have linked it to cytotoxic treatment, fluid retention secondary to renal failure and acute hypertensive crisis [1]. Differential diagnosis of PRES in patients with SLE should include: hypertensive encephalopathy, uremic encephalopathy, ischemic stroke and central nervous system activity.

The aims of the present study are to describe the clinical manifestations, risk factors, imaging characteristics and neurological outcome of PRES in patients with SLE.

Section snippets

Patients and clinical scores

The protocol was approved by our local committee of ethics. From October 2001 to January 2007, we identified those patients with clinical and imaging findings consistent with PRES in the emergency department and the inpatient section of our institution, which is a national tertiary reference center for patients with SLE and other immunological disorders. All patients had a previous diagnosis of SLE according to the American College of Rheumatology (ACR) criteria [8]; systemic activity was

Clinical findings

During the study period a total of 16,600 consultations were performed at the Emergency Room (ER) of our institution, from which 1425 (8.6%) were previous or newly diagnosis cases of SLE. We identified 21 episodes of PRES in 20 patients with SLE in the ER and 1 episode in the inpatient section of our institution. PRES represented 1.5% of all SLE consultations in the ER during the study period. The clinical and laboratory characteristics for the 22 episodes are shown in Table 1. At onset all

Discussion

The most accepted theory of PRES is a loss of autoregulation in cerebral blood-flow induced by hypertension with a subsequent increase in vascular permeability with leakage of blood plasma and erythrocites, producing vasogenic edema with petechial hemorrhages [1].

Patients with SLE may be at particular risk for this complication, because they may develop hypertension and fluid retention secondary to renal disease, and the use of cytotoxic drugs to control disease activity; besides, endothelial

Conclusions

The combination of high systemic activity, hypertension and renal failure may impose a compound risk for developing PRES in lupus patients. A established BBB damage may add an additional risk to develop PRES when systemic activity increases. The vasogenic edema may be the result of endothelial dysfunction, and although reversibility is common, this syndrome adds high acute morbidity and the possibility of clinical and radiological sequel. Although antihypertensive medication is considered the

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