Background/Purpose: Demyelinating syndromes (DS) in systemic lupus erythematosus (SLE) are characterized by inflammation, demyelination and neurodegeneration. Little is known, however, about the tenet of dissemination in time or space of DS in SLE. The aim of this study is to evaluate the long-term outcomes in SLE patients with DS in a large SLE cohort.

Methods: Data of patients with DS were obtained from the SLE cohort at the University of Maryland, between 1996 and 2016. Demographic, clinical features, serological studies, SLE-related treatment, and DS treatment-related exposures, Expanded Disability Status Scale (EDSS) score at baseline, were included. SLE patients with DS were classified as Clinically Isolated Syndrome (CIS), [those presenting with optic neuritis, partial myelitis or a brain stem syndrome], and Radiologically Isolated Syndrome (RIS), [those exhibiting radiological disease with T2/fluid attenuated inversion recovery or enhancing magnetic resonance imaging (MRI) typical of demyelination as a biomarker of acute inflammatory activity].The primary outcomes were DS progression, overall survival, cognitive dysfunction and residual disability. The predictors of DS progression were calculated using multivariable Cox proportional hazards regression analysis models. To avoid bias, patients were included in the analysis regardless of their duration of follow-up.

Results: 25 SLE cases with DS were identified [mean age; 41.9 + 12.3 years, 80 % African America, 84% women, with a mean follow up of 6.9 + 1.8 years]. The median baseline EDSS score was 4.5 (range, 1.5-6.5). CIS was observed in 15 cases (60 %) [Optic neuritis n=6 (24%), acute transverse myelitis n=9 (36%)]. Ten patients (40%) had RIS. Two patients (8 %) had neuromyelitis optica syndrome.

Nine patients (36%) had progressive forms of DS, 8 (32 %) had cognitive dysfunction and 9 (36 %) had worsening disability. Three deaths at follow up, were observed and were considered SLE-related mortality.

Factors associated with progression of DS included, SSA antibody [Odds ratio, 10.5 (95% CI: 1.5-72, p < 0.005)], and presence of oligoclonal bands on spinal fluid analysis. There were lack of association with INF gene expression, DS modifying therapy, phospholipid syndrome, or use of hydroxycholorquine.

Worsening disability overtime was associated with spinal cord lesions {OR 34 (95 % CI: 3.5-93.2, p < 0.001)], and CIS [OR 10.3 (95 % CI 1.0-102, p < 0.008)]. The use of DS modifying therapy tended to be protective, (OR 0.8, 95% CI, 0.6- 0.9, p < 0.051).

Older age (> 40 years) [OR 3.6 (95% CI: 1.7- 67, p < 0.03] and midbrain lesion (OR 2.7, 95 % CI: 1.1-6.5, p < 0.05)] were associated with cognitive impairment.

Conclusion: More than half of SLE patient with DS remained free from neurological progression for 5 years after DS diagnosis. Younger age, relapsing form of DS, prior immunotherapies, and lower baseline EDSS score were factors associated with better outcomes. Identifying novel biomarkers of DS progression in SLE will help develop therapeutic options and engender mechanisms of neurodegeneration.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-outcome-of-demyelnating-syndrome-in-systemic-lupus-erythematosus-a-longitudinal-study/