Abstract:

Objective To study the clinical profile of the posterior reversible encephalopathy syndrome (PRES) in children with systemic lupus erythematosus (SLE).Methods The analysis was based on 4 PRES patients with SLE admitted to our department (PUMCH) and 11 cases retrieved from PubMed to analyze the clinical manifestation, PRES features, laboratory abnormalities, treatment and outcomes.Results Firstly, 4 cases were diagnosed in our hospital, disease duration of SLE ranged from 1month to 63months. Nephritis was found in all these patients, and methylprednisolone and cyclophosphamide pulse therapy was administered in one patient. Four children with PRES presented seizures, headache and hypertension. Magnetic resonance showed predominantly posterior distribution involved. Secondly, 11 cases were retrieved from PubMed, together with our 4 cases, 15 cases (14 girls) were analyzed. The minimum age was 8 years. The average duration between SLE diagnosis and primary onset of PRES was 6 months (ranged from 1 month to 6 years). Clinical manifestations of PRES in children with SLE were seizures (all of 15 cases, 100.0%), headache (10/15, 66.7%), vomiting (7, 46.7%), loss of consciousness (9, 60.0%) and vision loss (7/15, 46.7%). Hypertension was seen in all 15 patients; 12 had nephritis; 4 were treated with methylprednisolone pulse therapy; 3 were treated with cyclophosphamide pulse therapy; 2 were treated with hydroxychloroquine; 1 was treated with cyclosporine A; 1 was treated with Rituximab. PRES occurred 2 days to 4years after immunosuppressive drugs therapy. All patients underwent MRI and the white matter of posterior region was mainly involved, and involvement in areas such as parietal lobes, brain stem and cerebellum was also found. 12 cases were treated with antihypertensive drugs. 10 cases were treated with anticonvulsant drugs in short duration. Glucocorticoid and immunosuppressive drug were used in 9 patients with active lupus, but they were withheld or reduced if the child was not accompanied by active lupus. All patients improved without neurological deficit. Follow up MRI showed marked improvement.Conclusion PRES in children with SLE presented seizures, headache, vomiting, loss of consciousness and vision loss. SLE patients with lupus nephritis, hypertension and use of immunosuppressive drug could precede the occurrence of PRES. Brain MRI is important for diagnosis of PRES. Early diagnosis, prompt control of blood pressure and seizure and appropriate use of glucocorticoid and immunosuppressive drug are keys to successful management of PRES.