Background/Purpose:  Posterior reversible encephalopathy syndrome (PRES) is a well-known but rare complication in systemic lupus erythematosus (SLE) patients (<1%). However, current epidemiologic data is quite scant. The aim of the present study was to describe potentially unrecognized risk factors.

Methods: We performed a single-center retrospective case-control study in a tertiary care center in Mexico City between 1999 and 2014. We included 48 patients (cases) with SLE diagnosis (≥4 ACR criteria) who presented with reversible neurological manifestations (seizures, visual abnormalities, acute confusional state, among others) associated with changes by magnetic resonance (MRI) (iso or hypointensity in T1 and hyperintensity in T2/FLAIR). Controls (n=96) were patients with SLE without evidence of PRES that were hospitalized during the same period as cases (± 3 months) and matched by gender. Association between variables was calculated by X² test and OR (95% CI). Multivariate analysis was performed by logistic regression.

Results: SLE patients with PRES were younger (27.9±1.05 vs 36.2±1.36 years, p˂0.001). Ninety percent of the cases occurred in women. PRES occurred in 28/48 patients (40%) after 24 hours of admission (2-30 days). The vast majority (80.2%) of cases presented with seizures, and up to 18% showed “atypical” MRI images. Decrease or resolution of MRI images in the first 12 weeks after the event occurred in 88.8% of cases.  Variables associated with the development of PRES, three months prior to hospitalization and at the time of the event are summarized in Table 1. After multivariate analysis, hypertension at admission [OR 16.3, 95% CI 4.03-65.85, p˂0.001], renal replacement therapy at discharge [OR 6.65, 95% CI 1.24-35.64, p=0.027], persistent lymphopenia (˂1,000 cells/uL in at least two consecutive measurement previous to the event) [OR 5.76, 95% CI 1.36-24.40, p=0.017], SLEDAI≥6 prior to admission [OR 1.11, 95% CI 1.01-1.22, p=0.031] and age [OR 0.863, 95% CI 0.81-0.91, p˂0.001] were independent risk factors for the development of PRES in SLE. Length of hospital stay was similar between groups (17.2±2.07 vs 14.60±1.11 days, p=0.26) and none of the cases died during hospitalization.

Conclusion: Our data is in agreement with prior studies that link end-stage renal disease, hypertension and high SLEDAI scores to the development of PRES in SLE. Furthermore, we found that persistent lymphopenia is a novel independent risk factor for PRES in SLE, which could be related to endothelial dysfunction in these patients.

Table 1.Variables associated with development of PRES in SLE patients (univariate analysis)

Three months prior to admission	OR	95% CI	p value
SLEDAI ≥6 points	4.41	1.87-10.40	<0.001
Hypertension	3.50	1.68-7.32	0.001
Lymphopenia	3.36	1.36-8.29	0.006
Low C3 levels	2.71	1.21-6.07	0.013
History of renal replacement therapy	2.66	1.15-6.12	0.019
At admission	OR	95% CI	p value
SLEDAI ≥6 points	33.80	4.43-257.54	<0.001
Hypertension	13.63	5.88-31.56	<0.001
GFR ≤60 mL/min/1.73 m2	4.75	1.93-11.65	<0.001
Renal replacement therapy at discharge	4.27	1.97-9.24	<0.001
Low C3 levels	2.22	1.02-4.81	0.041

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/risk-factors-for-posterior-reversible-encephalopathy-syndrome-in-systemic-lupus-erythematosus-patients-a-single-center-study/