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Objective

To analyze the clinical features and outcomes of patients with posterior reversible encephalopathy syndrome (PRES), the risk factors of PRES-related intracranial hemorrhage (ICH), and all-cause mortality in patients with systemic lupus erythematosus (SLE).

Methods

Twenty-six episodes of PRES were identified in 23 SLE patients, using an electronic medical records database of 3,746 SLE patients.

Results

The prevalence of PRES was 0.69% among SLE patients. The scores of the SLE Disease Activity Index without neurologic descriptors (SLEDAI-N) were significantly elevated from baseline for a mean of 3.3 during PRES (P = 0.009). Rapidly deteriorating renal function, pulmonary hemorrhage, thrombotic microangiopathy, macrophage activation syndrome, or multiple organ dysfunction syndrome appeared during 65.4% of episodes. In 16 episodes, patients completely recovered from PRES-related symptoms within a median of 7 days. Visual impairment was reversed within 2 days in 8 of 15 patients, but impairment in other patients was protracted for up to 4 months, especially when ICH was present. Hypoalbuminemia (<20 gm/liter; odds ratio [OR] 30, 95% confidence interval [95% CI] 2.04–441.84) and thrombocytopenia (<30,000/mm3; OR 21, 95% CI 1.27–346.93) were risk factors for PRES-related ICH. Patients with SLEDAI-N scores >18 during a PRES attack had significantly higher mortality rates than did patients with SLEDAI-N scores ≤18 (P = 0.009 by log rank test).

Conclusion

PRES frequently occurs during active SLE with multiple complications. Hypoalbuminemia and thrombocytopenia may contribute to PRES-related ICH. The extraneurologic disease activity of lupus during PRES may influence the mortality rate of SLE patients.