OBJECTIVE\nTo develop a standardized nomenclature system for the neuropsychiatric syndromes of systemic lupus erythematosus (NPSLE).\n\n\nMETHODS\nAn 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.\n\n\nRESULTS\nCase 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.\n\n\nCONCLUSION\nThe 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.

The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes

Objective

The frequency of coronary heart disease (CHD) and stroke are increased in systemic lupus erythematosus (SLE), but the extent of the increase is uncertain. We sought to determine to what extent the increase could not be explained by common risk factors.



Methods

The participants at two SLE registries were assessed retrospectively for the baseline level of the Framingham study risk factors and for the presence of vascular outcomes: nonfatal myocardial infarction (MI), death due to CHD, overall CHD (nonfatal MI, death due to CHD, angina pectoris, and congestive heart failure due to CHD), and stroke. For each patient, the probability of the given outcome was estimated based on the individual's risk profile and the Framingham multiple logistic regression model, corrected for observed followup. Ninety-five percent confidence intervals (95% CIs) were estimated by bootstrap techniques.



Results

Of 296 SLE patients, 33 with a vascular event prior to baseline were excluded. Of the 263 remaining patients, 34 had CHD events (17 nonfatal MIs, 12 CHD deaths) and 16 had strokes over a mean followup period of 8.6 years. After controlling for common risk factors at baseline, the increase in relative risk for these outcomes was 10.1 for nonfatal MI (95% CI 5.8–15.6), 17.0 for death due to CHD (95% CI 8.1–29.7), 7.5 for overall CHD (95% CI 5.1–10.4), and 7.9 for stroke (95% CI 4.0–13.6).



Conclusion

There is a substantial and statistically significant increase in CHD and stroke in SLE that cannot be fully explained by traditional Framingham risk factors alone.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/1529-0131%28200110%2944%3A10%3C2331%3A%3AAID-ART395%3E3.0.CO%3B2-I

Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus.

Objective

To determine rates of morbidity due to cardiovascular and cerebrovascular diseases among women with systemic lupus erythematosus (SLE).



Methods

I used the California Hospital Discharge Database, which contains information on all discharges from acute care hospitals in California, to identify women with SLE who had been hospitalized for treatment of either acute myocardial infarction (AMI), congestive heart failure (CHF), or cerebrovascular accident (CVA) from 1991 to 1994. I compared the proportions of hospitalizations for each cause among women with SLE with those in a group of women without SLE, for 3 age strata (18–44 years, 45–64 years, and ≥65 years).



Results

Compared with young women without SLE, young women with SLE were 2.27 times more likely to be hospitalized because of AMI (95% confidence interval [95% CI] 1.08–3.46), 3.80 times more likely to be hospitalized because of CHF (95% CI 2.41–5.19), and 2.05 times more likely to be hospitalized because of CVA (95% CI 1.17–2.93). Among middle-aged women with SLE, the frequencies of hospitalization for AMI and CVA did not differ from those of the comparison group, but the risk of hospitalization for CHF was higher (odds ratio [OR] 1.39, 95% CI 1.05–1.73). Among elderly women with SLE, the risk of hospitalization for AMI was significantly lower (OR 0.70, 95% CI 0.51–0.89), the risk of hospitalization for CHF was higher (OR 1.25, 95% CI 1.01–1.49), and the risk of hospitalization for CVA was not significantly different from those in the comparison group.



Conclusion

Young women with SLE are at substantially increased risk of AMI, CHF, and CVA. The relative odds of these conditions decrease with age among women with SLE.

Premature morbidity from cardiovascular and cerebrovascular diseases in women with systemic lupus erythematosus.

To the Editor:

We read with interest the article by Ainiala et al.1 describing the prevalence of neuropsychiatric syndromes in SLE. Neuropsychiatric syndromes may be present in patients with SLE (more than 50%), not only through serious manifestations such as seizures and psychosis but in other more subtle ways, such as cognitive impairment and mood disorder, even in neurologically or psychiatrically asymptomatic patients.2,3⇓ In this study, only 14 of 37 patients (38%) tested with cognitive dysfunction had cognitive problems. We studied 15 women with SLE in a control group with a mean age of 27.8 ± 6.35 years, mean disease duration since diagnosis of SLE of 4.8 ± 3.5 years, and mean level of education of 11.7 ± 6.3 years.4 At the time of the study, all patients were receiving low doses …

The prevalence of neuropsychiatric syndromes in systemic lupus erythematosus

Systemic Lupus Erythematosus (SLE) is defined by its clinical features and by the almost invariable presence in the blood of antibodies directed against one or more components of cell nuclei. Certa...

Systemic lupus erythematosus.

In a review of our experience with systemic lupus erythematosus (SLE) since 1975, we found 48 of 266 patients with major central nervous system (CNS) manifestations for which a non-SLE explanation could not be identified. Eleven patients developed more than one type of CNS event. The commonest symptom was seizure (18 patients), followed by brainstem dysfunction (12 patients), psychosis (11 patients), organic brain syndrome (11 patients) and stroke (7 patients). In 19% of cases, CNS manifestations were accompanied by a flare of multisystem SLE disease activity. Anticonvulsants were able to be discontinued safely in the majority of patients with seizures. Most CNS events were self-limited, reversible and not associated with poor outcome unless accompanied by multisystem disease activity. Therapy with corticosteroids did not appear to offer substantial benefit.

W P Olszynski

Papers

The incidence and prognosis of central nervous system disease in systemic lupus erythematosus.