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. 2013;8(2):e55843.
doi: 10.1371/journal.pone.0055843. Epub 2013 Feb 12.

Incidence and prevalence of major central nervous system involvement in systemic lupus erythematosus: a 3-year prospective study of 370 patients

Eleni I Kampylafka  1 , Haralampos Alexopoulos, Michalis L Kosmidis, Demosthenes B Panagiotakos, Panayiotis G Vlachoyiannopoulos, Marinos C Dalakas, Haralampos M Moutsopoulos, Athanasios G Tzioufas
Affiliations

Incidence and prevalence of major central nervous system involvement in systemic lupus erythematosus: a 3-year prospective study of 370 patients

Eleni I Kampylafka et al. PLoS One. 2013.

Abstract

Background: The 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.

Methods: 370 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.

Results: 16/370 (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).

Conclusions: Clinically 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.

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Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Numbers of patients with the respective antibodies within each CNS subgroup.
Each column group refers to the respective CNS subgroup. Each column represents a discrete autoantibody, while Y axes displays the number of patients presenting with the respective autoantibody.
See this image and copyright information in PMC

References

    1. D’Cruz DP, Khamashta MA, Hughes GR (2007) Systemic lupus erythematosus. Lancet 369: 587–596. - PubMed
    1. Bernatsky S, Clarke A, Gladman DD, Urowitz M, Fortin PR, et al. (2006) Mortality related to cerebrovascular disease in systemic lupus erythematosus. Lupus 15: 835–839. - PubMed
    1. Hanly JG, Urowitz MB, Sanchez-Guerrero J, Bae SC, Gordon C, et al. (2007) Neuropsychiatric events at the time of diagnosis of systemic lupus erythematosus: an international inception cohort study. Arthritis Rheum 56: 265–273. - PubMed
    1. Hanly JG, Urowitz MB, Su L, Bae SC, Gordon C, et al. (2010) Prospective analysis of neuropsychiatric events in an international disease inception cohort of patients with systemic lupus erythematosus. Ann Rheum Dis 69: 529–535. - PMC - PubMed
    1. Bosnic D, Cerovec M, Anic B, Mayer M, Sentic M, et al. (2008) [Cryptococcal meningitis as a diagnostic problem in a patient with SLE–case report]. Lijec Vjesn 130: 136–140. - PubMed

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