Background/Purpose: Neuropsychiatric systemic lupus erythematosus (NPSLE) is a serious organ disorder with a variety of symptoms. Despite advances in the understanding of the immunopathogenic and clinical aspects of SLE, NPSLE remains a diagnostic and therapeutic challenge. The therapeutic outcomes among very few published controlled studies are diverse, because of the variability in the manifestations of NPSLE and the absence of appropriate diagnostic criteria.

Methods: This study was conducted to investigate the immunopathogenic and clinical aspects and treatment outcomes of NPSLE. We analyzed the laboratory data, symptoms, treatment regimen, and therapeutic outcomes 1 year after treatment, and the prognostic factors of 28 NPSLE patients admitted to our hospital in an 8-year period from 2006 through 2013 and 27 cytokine, chemokines and growth factor profiles in pretreatment samples of their cerebrospinal fluid (CSF) using the Bio-Plex Human 27-plex panel.

Results: There were 26 females (92.9%) and two male. The median age at the onset of NPSLE was 35 years, ranging from 15 to 53 years old. The median duration from SLE onset to first neuropsychiatric event was 7 years. The median Safety of Estrogens in Lupus Erythematosus National Assessment–Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) score at the disease onset of NPSLE was 13. Twelve patients were responders at 1 year post-treatment; their median age at NPSLE onset was 29 years versus 39 years in the non-responders (n=16, p=0.0260). The median duration from SLE onset of NPSLE was 1.5 years in responder versus 11 years in non-responder (p=0.0096). Patients with more than two NPSLE symptom types had significantly poorer outcomes (p=0.0159). The CSF interleukin (IL)-10, interferon (IFN)-γ and tumor necrosis factor (TNF)-α levels before the treatment were significantly higher in the non-responders (p=0.0051, p=0.0239 and p=0.0258, respectively). Ranking of the cytokines/chemokines to distinguish responder from non-responder by weighted-voting algorithm showed that the combination of IL-10, TNF-α, IL-6, IFN-γ, IL-4 and IL-13 had a highest Matthews correlation coefficient.

Conclusion: Younger, shorter disease duration and single-symptom NPSLE patients had significantly better therapeutic outcomes. The lower cytokine value of IL-10, IFN-γ and TNF-α before the treatment in CSF may provide better NPSLE outcomes. Additionally, measurement of multiple cytokines in pretreatment such as IL-10, TNF-α, IL-6, IFN-γ, IL-4 and IL-13 could distinguish responder from non-responder patients. Our findings may suggest the importance of making a diagnosis at an earlier phase for better therapeutic response and measuring multiple cytokines to predict therapeutic outcomes of NPSLE.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/predictors-of-therapeutic-outcomes-in-patients-with-neuropsychiatric-systemic-lupus-erythematosus/