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

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.

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…Epileptic seizures are a common CNS manifestation, occurring in as much as 2-10 % of patients, mainly associated with high disease activity and antiphospholipid antibodies [5,23,28]. Apart from symptomatic antiepileptic therapy, immunosuppression is indicated when high disease activity coexists or whenever there is suspicion of underlying inflammatory m e c ha n i s m s ( g r ad e 3 D ) ; w h e t h er t he p r op o s e d hydroxychloroquine has a protective effect (grade 2C) remains to be determined [23].…”

“…The main risk factors are cumulative damage attributed to the systemic disease and antiphospholipid antibodies [5,28], necessitating therapy with anticoagulants or antiplatelet agents (grade 2C), along with adequate control of non-SLE-related risk factors. Dopamine antagonists may be used in persistent chorea (grade 3D) [22,29].…”

Systemic Lupus Erythematosus with Neuromyelitis Optica

“…Neuropsychiatric SLE (NPSLE) refers to several different neurological and/or behavioral clinical syndromes such as stroke, seizures, myelopathy, mood disorders, and cognitive impairment [1] (Table 1), and a 30-40% prevalence rate of NPSLE has recently been reported [2]. In a 3-year prospective study of 370 patients with SLE and no history of CNS complication, 16/370 patients were reported to develop major CNS-related events including myelopathy (4/16), optic neuritis (1/16), and positive neuromyelitis optica (NMO)-IgG antibodies (2/16) [3]. It has been shown that antiphospholipid antibodies including anti-cardiolipin and lupus anti-coagulant are some of the strongest NPSLE-related factors expressed especially in relation to cerebrovascular disease [4,5].…”

“…As discussed, anti-AQP4 antibodies have been discovered both in patients with NMO and with NPSLE. Table 1: Major manifestations of CNS lupus [3].…”

Headache, Seizures, Cognitive Disorders, and Cerebrovascular Diseases as the Most Common Neuropsychic Syndromes in Systemic Lupus Erythematosus

“…тонико-клонических припадков, у 3 пациентов был эпилептический статус [41]. У 5-10% больных судороги возникают за несколько лет до появления других признаков СКВ [39,42].…”