No abstract

Objective. To develop a standardized nomenclature system for the neuropsychiatric syndromes of systemic lupus erythematosus (NPSLE).Methods. An international, multidisciplinary committee representing rheumatology, neurology, psychiatry, neuropsychology, and hematology developed case definitions, reporting standards, and diagnostic testing recommendations. Before and after the meeting, clinician committee members assigned diagnoses to sets of vignettes randomly generated from a pool of 108 NPSLE patients. To assess whether the nomenclature system improved diagnostic agreement, a consensus index was developed and pre-and postmeeting scores were compared by t-tests.Results. Case definitions including diagnostic criteria, important exclusions, and methods of ascertainment were developed for 19 NPSLE syndromes. Recommendations for standard reporting requirements, minimum laboratory evaluation, and imaging techniques were formulated. A short neuropsychological test battery for the diagnosis of cognitive deficits was proposed. In the postmeeting exercise, a statistically significant improvement in diagnostic agreement was observed.Conclusion. The American College of Rheumatology (ACR) Nomenclature for NPSLE provides case definitions for 19 neuropsychiatric syndromes seen in SLE, with reporting standards and recommendations for laboratory and imaging tests. It is intended to facilitate and enhance clinical research, particularly multicenter studies, and reporting. In clinical settings, consultation with other specialists may be required. It should be useful for didactic purposes but should not be used uncritically or as a substitute for a clinical diagnosis. The complete case definitions are available on the ACR World Wide Web site: http://www.rheumatology .org/ar/ar.html.

Eighty-six females with systemic lupus erythematosus (SLE) were grouped according to present or past history of neuropsychiatric (NP) symptomatology (Active, Inactive, or Never). Performance of these three groups was compared to that of 35 normal women on an extensive battery of neuropsychological tests sampling a wide range of cognitive functions. In addition to making group comparisons, we also devised a system for identifying individual impairment using decision rules for both quantitative and qualitative data. Our results indicate that a variety of cognitive deficits are present in SLE patients taken together as a group; there is no significant association between cognitive impairment and emotional disturbance; patients with resolved NP symptomatology are as impaired as patients with active NP symptoms, suggesting residual CNS involvement; in spite of no significant difference emerging on direct group comparisons, significantly more Never NP-SLE patients are impaired than are controls on several summary scores, suggesting subclinical CNS involvement in these patients.

Objective. This study was designed to assess the effects of corticosteroids on select aspects of nervous system functioning, specifically, cognition and mood, as well as disease-related symptoms in individual patients with mild systemic lupus erythematosus (SLE) and mild neuropsychiatric ( N P ) symptoms.Methods. Ten women who had not been taking corticosteroids for at least 6 months were selected from a referral-based lupus clinic to participate in an N of 1 double-blind, controlled trial consisting of 3 randomly assigned drug/placebo pairings, with a drug dose of 0.5 mgkg of prednisone daily.Results. Analysis of variance on the group data yielded significant positive drug effects for cognition (P = 0.02), mood (P = 0.003), and SLE symptom ratings (P = 0.0002). Drug efficacy was also evaluated by an objective decision rule, which yielded evidence of overall drug benefit in 5 of the 8 patients who completed the trial, and a deleterious drug effect in 1 patient. Posttrial clinical results indicated that for the 8 women who completed the trial, "acceptable" decisions, leading to remission of SLE symptoms or appropriate withholding of steroids, were made on the basis of this rule. Conclusion. Improvement in cognition, mood, and/or SLE symptom ratings can be observed following brief exposure to relatively low doses of corticosteroids in individual women with mild SLE; these persist over repeated drug exposure. The current application of N of 1 methodology represents the first systematic study of

The pathogenesis of neuropsychiatric lupus (NP-SLE) is unclear, but may involve vasculopathy, antibodies against nervous system tissue, or both. A major difficulty in determining the significance of antineuronal antibodies in NP-SLE has been lack of consistent clinical diagnostic approaches. By utilizing a new clinical classification of NP-SLE, neuropsychological assessments, and an assay for IgG antineuronal antibodies, we have found a significant association between antibody-positivity and cognitive impairment or nonfocal NP-SLE. These observations indicate that antineuronal antibodies may play a role in NP-SLE and emphasize the clinical importance of cognitive function in patients with SLE.