Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Acute Flares of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) Are Sometimes Associated with Spikes of Interferon-alpha Activity in the CSF.

Svenungsson2,  Elisabet, Mavragani1,  Clio P., Hopia3,  Liisa, Khademi3,  Mohsen, Laveskog2,  Anna, Crow1,  Mary K., Andersson2,  Magnus

Hospital for Special Surgery, New York, NY
Karolinska Institutet, Stockholm, Sweden
Karolinska Institutet, Sweden


Neuropsychiatric involvement in Systemic Lupus Erythematosus (NPSLE) is a serious complication affecting a large proportion of systemic lupus erythematosus (SLE) patients. Diagnosis and treatment is a challenge as no specific biomarker/investigation for NPSLE is available. Enhanced interferon-alpha (IFN-a) activity has been identified as an important ethiopathogenic factor in SLE, and a previous study reported that IFN-a contributes to NPSLE. We investigated IFN-a activity in the systemic and intrathecal compartments of patients with acute and chronic symptoms of NPSLE.


59 NPSLE patients, who fulfilled the ACR 1982 revised criteria for SLE, were evaluated clinically. Lumbar puncture and magnetic resonance imaging (MRI, n=56) was performed. All presented with one or several NPSLE manifestations as defined by the ACR NPSLE case definitions. The majority (n= 49) had chronic NPSLE symptoms but 10 were investigated during acute NPSLE flares. Paired blood and cerebrospinal fluid (CSF) samples were collected from patients and controls (15 Multiple Sclerosis (MS) patients, 22 patients with other neurological diseases (OND)). IFN-a activity was measured as a score with a functional cell reporter assay. mRNA expression of IFN-a was determined in peripheral blood and CSF mononuclear cells (PBMC, CSF-MC) in 39 patients and in a different set of controls (MS=34, OND=24).


On a group level there were no differences between SLE patients and controls with respect to IFN-a activity or IFN-a mRNA expression in PBMCs or CSF-MCs, but IFN-a scores were higher in acute than in chronic NPSLE cases (p=0.007). A few patients, but no controls, had very high levels of IFN-a activity in CSF and blood. 4/10 acute vs. 5/49 chronic NPSLE patients had high (>+2SD) IFN-a activity in the CSF (p=0.02). A similar trend was observed in blood where 5/10 with acute vs. 5/49 with chronic NPSLE had high IFN-a scores (p=0.07). There was no corresponding increase in mRNA expression in CSF-MCs or PBMCs. Two patients had very high mRNA expression in CSF-MCs. One of these patients had an acute NPSLE flare. There was no specific NPSLE symptom (mood disorder, headache, cognitive dysfunction, seizures or previous infarction), CSF aberration or MRI finding, which was convincingly associated with high IFN-a activity or high expression of IFN-a mRNA.


Our results indicate that IFN-a activity in the CSF is high in some patients with NPSLE and is often associated with acute NPSLE symptoms. As a group, patients with chronic NPSLE had similar IFN-a activity in CSF and blood to controls. IFN-a activity may be of diagnostic potential in patients with SLE who present with acute neurological symptoms.

To cite this abstract, please use the following information:
Svenungsson, Elisabet, Mavragani, Clio P., Hopia, Liisa, Khademi, Mohsen, Laveskog, Anna, Crow, Mary K., et al; Acute Flares of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) Are Sometimes Associated with Spikes of Interferon-alpha Activity in the CSF. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1181
DOI: 10.1002/art.28947

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