Abstract
BackgroundThe incidence and prevalence of CNS involvement in SLE remains unclear owing to conflicting results in the published studies. The aim of the study was to evaluate the incidence and prevalence of major definite CNS events in SLE patients.Methods370 SLE patients with no previous history of CNS involvement were prospectively evaluated in a tertiary hospital referral center for 3 years. Major CNS manifestations were codified according to ACR definitions, including chorea, aseptic meningitis, psychosis, seizures, myelopathy, demyelinating syndrome, acute confusional state and strokes. Minor CNS events were excluded. ECLAM and SLEDAI-SELENA Modification scores were used to evaluate disease activity and SLICC/ACR Damage Index was used to assess accumulated damage.Results16/370 (4.3%) patients presented with a total of 23 major CNS events. These included seizures (35%), strokes (26%), myelopathy (22%), optic neuritis (8.7%), aseptic meningitis (4.3%) and acute psychosis (4.3%). Incidence was 7.8/100 person years. Among hospitalizations for SLE, 13% were due to CNS manifestations. Epileptic seizures were associated with high disease activity, while myelopathy correlated with lower disease activity and NMO-IgG antibodies (P≤0.05). Stroke incidence correlated with APS coexistence (P = 0.06). Overall, CNS involvement correlated with high ECLAM and SLEDAI scores (P<0.001).ConclusionsClinically severe CNS involvement is rare in SLE patients, accounting for 7.8/100 person years. CNS involvement correlates with high disease activity and coexistence of specific features that define the respective CNS syndromes.
Highlights
Systemic Lupus Erythematosus (SLE) is an autoimmune systemic disease which has a prevalence of 52.2/100.000 in the United States [1]
After excluding the non-specific minor Central Nervous System (CNS) complaints (n = 76 patients), the prevalence of major CNS events among SLE patients was 4.3%; 16/370 patients presented a total of 23 CNS events
All seizures were generalized tonic– clonic, while 3 patients evolved to status epilepticus
Summary
Systemic Lupus Erythematosus (SLE) is an autoimmune systemic disease which has a prevalence of 52.2/100.000 in the United States [1]. Formulated a nomenclature system which provided case definitions for 19 neuropsychiatric SLE syndromes[8] Despite these advances, the reported frequency of neurologic involvement in SLE continues to vary greatly, ranging from 12% to 95% [9,10,11,12]. The reported frequency of neurologic involvement in SLE continues to vary greatly, ranging from 12% to 95% [9,10,11,12] This discrepancy can be attributed to factors such as study design, diagnostic approach and patients’ baseline characteristics [10,12]. The aim of the study was to evaluate the incidence and prevalence of major definite CNS events in SLE patients
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