Dataset Information

Neuropsychiatric features of a cohort of patients with systemic lupus erythematosus.

ABSTRACT: In order to establish if neuropsychiatric systemic lupus erythematosus (NPSLE) can be identified by any characteristic other than those used to diagnose the neuropsychiatric (NP) disease itself, we retrospectively reviewed 98 systemic lupus erythematosus (SLE) patients followed over a mean period of 10 years. NPSLE was identified in 22 patients. Stroke and generalized seizures were the most frequent NP manifestations. The NPSLE and non-NPSLE groups were similar with regard to demographic characteristics, ACR criteria, serum autoantibodies, and frequency of hypertension and hypercholesterolemia. Of note, compared to the non-NPSLE group, NPSLE was associated with a higher frequency of smoking (78 versus 26%), organ damage (73 versus 34%), and cumulative mortality rate (14 versus 7%). The series of patients was further analysed according to the presence of antiphospholipid syndrome (APS). Significantly, the interval between the onset of NP disease and SLE diagnosis was shorter in the APS(-) (0.3 ± 1 years) than in the APS(+) (5 ± 7 years) groups. Recurrence and/or persistence of NP events were only documented in the APS(-) group. Overall cumulative mortality was highest in NPSLE and in APS(+) patients with inadequate anticoagulation control, identifying an aspect that requires improved vigilance and the development of novel therapeutic modalities.

SUBMITTER: Moraes-Fontes MF

PROVIDER: S-EPMC3512311 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature

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Neuropsychiatric features of a cohort of patients with systemic lupus erythematosus.

Moraes-Fontes Maria Francisca MF Lúcio Isabel I Santos Céu C Campos Maria Manuel MM Riso Nuno N Vaz Riscado Manuel M

ISRN rheumatology 20121120

In order to establish if neuropsychiatric systemic lupus erythematosus (NPSLE) can be identified by any characteristic other than those used to diagnose the neuropsychiatric (NP) disease itself, we retrospectively reviewed 98 systemic lupus erythematosus (SLE) patients followed over a mean period of 10 years. NPSLE was identified in 22 patients. Stroke and generalized seizures were the most frequent NP manifestations. The NPSLE and non-NPSLE groups were similar with regard to demographic charact ...[more]

PMID: 23227358

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Project description:BackgroundIn clinical practice, discrimination between central nervous system (CNS) infections in patients with systemic lupus erythematosus (SLE) and neuropsychiatric lupus erythematosus (NPSLE) could be urgent and critical yet extremely challenging. Given this, this study aimed to investigate the clinical features and outcomes of infections in the CNS in patients with SLE and to establish a simplified scoring system for guiding the discrimination of CNS infections from NPSLE.MethodsA total of 95 patients who were identified as having CNS infections among 8491 SLE patients between January 1992 and January 2018 were included in this retrospective study. NPSLE patients admitted at the same period were randomly selected for comparison. Key factors either clinically valuable or statistically significant for discriminating CNS infections from NPSLE were integrated to build a simplified scoring system. Another group of 22 SLE patients complicated with suspected newly onset of CNS infections or NPSLE admitted after January 2018 was enrolled to verify the utility of the scoring system.ResultsSixty-three positive pathogens were identified in 59 patients of the total 95 CNS infection cases. Compared with the NPSLE group, the CNS infections group had a longer disease duration (21.0 [3.0-50.0] vs. 1.0 [0-22.0] months, P < 0.05), exhibited more fever (96.8% vs. 23.2%, P < 0.001) and polymorphonuclear leukocyte leukocytosis in the cerebrospinal fluid (CSF) (45.6% vs. 0.5%, P < 0.05), and had significantly decreased CSF glucose (2.0 ± 1.3 vs. 3.3 ± 0.9 mmol/L, P < 0.01), whereas hypocomplementemia seemed to be a strong hint of NPSLE (44.6% vs. 77.4%, P < 0.001). A simplified scoring system integrated with 8 key factors was established for guiding clinical differential diagnosis. By setting the cutoff value at 4 and verifying in a group of SLE patients complicated with newly occurred suspected CNS infection or NPSLE, a sensitivity of 85.7% and specificity of 93.3% with the area under the curve (AUC) being 0.93 (95%CI 0.80-1.00) were obtained.ConclusionsCNS infections are a fatal complication of SLE and can be difficult to discriminate from NPSLE. A simplified scoring system may help to make preliminary discrimination of CNS infections from NPSLE.

Project description:Neuropsychiatric systemic lupus erythematosus (NPSLE) is a generic definition referring to a series of neurological and psychiatric symptoms directly related to systemic lupus erythematosus (SLE). NPSLE includes heterogeneous and rare neuropsychiatric (NP) manifestations involving both the central and peripheral nervous system. Due to the lack of a gold standard, the attribution of NP symptoms to SLE represents a clinical challenge that obligates the strict exclusion of any other potential cause. In the acute setting, management of these patients does not differ from other non-SLE subjects presenting with the same NP manifestation. Afterwards, an individualized therapeutic strategy, depending on the presenting manifestation and severity of symptoms, must be started. Clinical trials in NPSLE are scarce and most of the data are extracted from case series and case reports. High-dose glucocorticoids and intravenous cyclophosphamide remain the cornerstone for patients with severe symptoms that are thought to reflect inflammation or an underlying autoimmune process. Rituximab, intravenous immunoglobulins, or plasmapheresis may be used if response is not achieved. When patients present with mild to moderate NP manifestations, or when maintenance therapy is warranted, azathioprine and mycophenolate may be considered. When symptoms are thought to reflect a thrombotic underlying process, anticoagulation and antiplatelet agents are the mainstay of therapy, especially if antiphospholipid antibodies or antiphospholipid syndrome are present. Recent trials on SLE using new biologicals, based on newly understood SLE mechanisms, have shown promising results. Based on what we currently know about its pathogenesis, it is tempting to speculate how these new therapies may affect the management of NPSLE patients. This article provides a comprehensive and critical review of the literature on the epidemiology, pathophysiology, diagnosis, and management of NPSLE. We describe the most common pharmacological treatments used in NPSLE, based on both a literature search and our expert opinion. The extent to which new drugs in the advanced development of SLE, or the blockade of new targets, may impact future treatment of NPSLE will also be discussed.

Dataset Information

Neuropsychiatric features of a cohort of patients with systemic lupus erythematosus.

Publications

Neuropsychiatric features of a cohort of patients with systemic lupus erythematosus.

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