Posterior reversible encephalopathy syndrome in Korean patients with systemic lupus erythematosus: risk factors and clinical outcome

Abstract

Posterior reversible encephalopathy syndrome (PRES) is an uncommon neurologic condition associated with systemic lupus erythematosus (SLE). This study aimed to demonstrate the risk factors and clinical outcome of PRES in patients with SLE. Fifteen patients with SLE were diagnosed with PRES by characteristic clinical manifestations and magnetic resonance imaging (MRI) features from 2000 to 2012. Clinical profiles and outcomes were assessed for this study population. Additionally, 48 SLE patients with neurologic symptoms who underwent brain MRI were included for comparative analyses. The median age and duration of SLE in patients with PRES was 27 and 6.1 years, respectively. Comparison between patients with and without PRES revealed significant differences in the presentation of hypertension and seizure, lupus nephritis with renal insufficiency, treatment with high-dose steroid and cyclophosphamide, recent transfusion, and lupus activity measured by SLE disease activity index. Renal failure was the single independent factor with a high odds ratio of 129.250 by multivariate analysis. Of 15 patients, four experienced relapse and two died of sepsis during hospitalization. Our results suggest that lupus nephritis with renal dysfunction and other related clinical conditions can precede the occurrence of PRES in patients with SLE. It is important to perform early brain imaging for a timely diagnosis of PRES when clinically suspected.

Clinical outcomes and risk factors for posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a multicentric case–control study

“…Although the prevalence of PRES in patients with SLE has been reported to be less than 1%,21 SLE is the most frequently associated rheumatological disease 14. Aggressive immunosuppressive treatments, high SLEDAI scores, renal dysfunction and uncontrolled hypertension have been previously described as factors related to PRES development in patients with SLE 10 11 13. In this study, the largest to date evaluating SLE-associated PRES, we corroborated the presence of all those variables as independent risk factors, and also described two novel-associated features: lymphopenia and dyslipidemia.…”

“…Up to 50% of patients with SLE may present neurological signs or symptoms throughout their disease,16 and while 19 neuropsychiatric syndromes have been described in SLE,17 PRES is not one of them. Lupus nephritis,13 hypertension,11 high activity scores according to Systemic Lupus Erythematosus Activity Index (SLEDAI)18 and the recent use of immunosuppressive drugs,12 have been described as the most relevant risk factors for the SLE/PRES association. It is possible that there are other specific risk factors, immunological and metabolic, for the development of PRES in patients with SLE.…”

Imaging characteristics associated with clinical outcomes in posterior reversible encephalopathy syndrome

“…[4, 1, 5] Alternatively, inflammation-mediated endothelial dysfunction may play a role in the development of PRES. [6] PRES is associated with autoimmune disorders,[7, 8] bone marrow and solid organ transplantation, [9–11] cancer,[12] sepsis,[13] and the peripartum state. [14…”

Corticosteroid therapy and severity of vasogenic edema in posterior reversible encephalopathy syndrome

“…PRES is associated with autoimmune disorders [10, 21], bone marrow and solid organ transplantation [16, 39, 40], cancer [43], sepsis [3], and the peripartum state [4]. Corticosteroids are frequently used in many of these settings.…”