Long-term outcome of status epilepticus-related to systemic lupus erythematosus: An observational study and a systematic review

Abstract

Objectives: Status epilepticus-related to systemic lupus erythematosus (SE-SLE) is in general attributed to fulminate neuropsychiatric lupus disease activity, yet the long-term outcome of SE-SLE is not well recognized. This is an observational study of 40 SE-SLE patients pooled from 8 cases at a single tertiary care hospital, and 32 SE-SLE patients identified on a systematic review, with focus on electro-clinical characteristics, imaging studies and the underlying etiology of SE-SLE in correlation with long-term outcome.

Results: Clinical phenotypes of SE-SLE were heterogeneous, ranging from patients with aura continua to patients in coma. Convulsive SE-SLE occurred among patients with heightened global lupus disease activity and increased cortical and subcortical brain lesion burden localized mostly in the frontal and temporal regions. There were no specific neuroimaging or laboratory abnormalities that allowed early SE-SLE diagnosis where a cluster of cases were of unclear etiology (17.5%). Most SE-SLE cases evolved to refractory SE-SLE with resistance to multiple anti-seizure medications and intravenous anesthetics requiring aggressive immune therapy that led to resolution of SE-SLE active phase. Seizure freedom occurred in 60.0% of patients and the median time to cessation of SE-SLE seizure activity after aggressive therapy was 14 days. Poor long-term outcomes were apparent in SE-SLE patients with one-year mortality (12.5%), recurrent SE-SLE (25.0%), subsequent epilepsy (37.5.1%), poor functional outcome (55.0%) and cognitive impairment (47.5%). A prolonged time to cessation of SE-SLE seizure activity was associated with unfavorable long-term outcome.

Conclusions: Diagnostic accuracy of SE-SLE requires better understanding of the etio-pathogenesis and the spectrum of clinical presentations of SE-SLE. Prompt initiation of immune therapy improve SE-SLE outcome, yet optimal therapeutic strategies remain to be determined. Identifying novel biomarkers that distinguish between different forms of SE-SLE and target cellular inflammatory response will help with specific SE-SLE treatment guidelines and prevent poor outcome.

Keywords: Clinical long-term outcome; Refractory status epilepticus; SLE-related status epilepticus; Systematic review; Systemic lupus, Autoimmune epilepsy.