Clinical features of reversible posterior leukoencephalopathy syndrome in patients with systemic lupus erythematosus

Abstract

To characterize reversible posterior leukoencephalopathy syndrome (RPLS) in systemic lupus erythematosus (SLE) in terms of treatments for resolution and its clinical course, we reviewed 28 cases of RPLS in SLE including our cases in view of the treatment. Of these, 15 cases improved with blood pressure control and 13 required immunosuppressive therapy for activity of SLE presenting neurological manifestations. Patients without immunosuppressants at onset of RPLS more frequently required immunosuppressive therapy to recover it than those precedingly using these agents [31% (4/13) versus 87% (13/15), p = 0.008, chi-square test]. Brain magnetic resonance imaging (MRI) is important for diagnosis of RPLS-SLE in the patient with SLE who develops neurological disturbance and rapidly increasing blood pressure. When 7-day therapy for hypertension and convulsion does not reverse the manifestations, immunosuppressive treatments would be recommended to reverse RPLS.

Incidence and Prevalence of Major Central Nervous System Involvement in Systemic Lupus Erythematosus: A 3-Year Prospective Study of 370 Patients

“…Three out of 6 patients who manifested epileptic seizures had characteristic features in their MRIs; two had Posterior Reversible Encephalopathy Syndrome (PRES) [44] and 1 had vasculitic-like lesions. All 3 patients had common clinical features; very high disease activity, active glomerulonephritis presenting as nephrotic syndrome, and symptom amelioration following immunosuppression and antiepileptic treatment.…”

“…All 3 patients had common clinical features; very high disease activity, active glomerulonephritis presenting as nephrotic syndrome, and symptom amelioration following immunosuppression and antiepileptic treatment. According to previous reports, two types of PRES in SLE are thought to exist; “hypertensive PRES,” being reversible with antihypertensive and anticonvulsive treatment concomitant with inactive SLE, and PRES requiring immunosuppressive therapy that could be considered a neurological manifestation of active SLE [44]. Finally, as seizures preceded the onset of the nephrotic syndrome, it is unlikely that they were due to nephrotic encephalopathy.…”

Parainfluenza virus infection associated with posterior reversible encephalopathy syndrome: a case report

“…To date, there have been two types of "PRES-SLE" syndromes described: "hypertensive PRES," which is reversible with conventional antihypertensive and anticonvulsive treatments concomitant with inactive SLE, and "immunological PRES," which requires immunosuppressive therapy and is considered a neurological manifestation of active SLE [12]. Our patient likely had the former type of PRES-SLE, given the fact that markers of lupus activity were normal and her clinical findings were not consistent with cerebral vasculitis or a systemic disease process.…”

“…Treatment of PRES generally includes antihypertensive and/or anticonvulsant therapies and the withdrawal of a suspected drug if applicable [17]. When seven-day therapy for hypertension and convulsion does not reverse the manifestation, immunosuppressive treatments are recommended to reverse PRES [12]. Recurrence of atypical PRES in a hypertensive child with ESRD on peritoneal dialysis was reported by Girişgen et al , and they noted infection and sudden increases in blood pressure as causes for recurrence [18].…”

Differential serum cytokine profile in patients with systemic lupus erythematosus and posterior reversible encephalopathy syndrome

“…Regarding the latter, our group recently described the main independent risk factors associated with the development of PRES in SLE patients, including lymphopenia and dyslipidaemia, which could contribute to endothelial dysfunction . Also, it has been proposed that, in SLE subjects, PRES can be associated either with blood pressure dysregulation or with an immune‐mediated aetiology . However, evidence supporting the potential mechanisms associated with PRES is quite scarce.…”