The American College of Rheumatology Nomenclature and Case Definitions forNeuropsychiatric Lupus Syndromes

Objective. To develop a standardized nomenclature system for the neuropsych iatric syndromes of systemic lupus erythematosus (NPSLE). Methods. An international, multidisciplinary committee representing rheumat ology, neurology, psychiatry, neuropsychology, and hematology developed cas e definitions, reporting standards, and diagnostic testing recommendations. Before and after the meeting, clinician committee members assigned diagnos es to sets of vignettes randomly generated from a pool of 108 NPSLE patient s. To assess whether the nomenclature system improved diagnostic agreement, a consensus index was developed and pre- and postmeeting scores were compa red by t-tests. Results. Case definitions including diagnostic criteria, important exclusio ns, and methods of ascertainment were developed for 19 NPSLE syndromes. Rec ommendations for standard reporting requirements, minimum laboratory evalua tion, and imaging techniques were formulated. A short neuropsychological te st battery for the diagnosis of cognitive deficits was proposed. In the pos tmeeting exercise, a statistically significant improvement in diagnostic ag reement was observed. Conclusion. The American College of Rheumatology (ACR) Nomenclature for NPS LE provides case definitions for 19 neuropsychiatric syndromes seen in SLE, with reporting standards and recommendations for laboratory and imaging te sts. It is intended to facilitate and enhance clinical research, particular ly multicenter studies, and reporting. In clinical settings, consultation w ith other specialists may be required. It should be useful for didactic pur poses but should not be used uncritically or as a substitute for a clinical diagnosis. The complete case definitions are available on the ACR World Wi de Web site: http//www.rheumatology.org/ar/ar.html.