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

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.

Definition of NPSLE: Does the ACR Nomenclature Still Hold?

“…In 1999, the ACR research committee, composed by experts from a variety of subspecialties including rheumatology, neurology, immunology, psychiatry and neuropsychology produced a standard nomenclature and set of case definitions for NPSLE (28, 29). Nineteen NP syndromes (Tables 1, 2) were defined and diagnostic criteria, criteria for exclusion, and specific diagnostic tests (laboratory and imaging evaluation) were defined (28, 29).…”

“…Nineteen NP syndromes (Tables 1, 2) were defined and diagnostic criteria, criteria for exclusion, and specific diagnostic tests (laboratory and imaging evaluation) were defined (28, 29). The new ACR nomenclature system was intended to expand the neuropsychiatric criteria of the ACR classification criteria for SLE (23, 28–30).…”

“…Nineteen NP syndromes (Tables 1, 2) were defined and diagnostic criteria, criteria for exclusion, and specific diagnostic tests (laboratory and imaging evaluation) were defined (28, 29). The new ACR nomenclature system was intended to expand the neuropsychiatric criteria of the ACR classification criteria for SLE (23, 28–30). A patient could be considered to have NPSLE, if they met the case definition for neuropsychiatric lupus and in addition met 3 or more of the ACR (non-NPSLE) criteria for SLE (28, 29).…”

“…The new ACR nomenclature system was intended to expand the neuropsychiatric criteria of the ACR classification criteria for SLE (23, 28–30). A patient could be considered to have NPSLE, if they met the case definition for neuropsychiatric lupus and in addition met 3 or more of the ACR (non-NPSLE) criteria for SLE (28, 29). However, the ACR classification criteria were not elaborated to replace clinical judgment or intended to make a clinical diagnosis in a given patient (28, 29).…”

Cavum Vergae, Liability, and Steroid Treatment: Manic Episode, Brain Imaging Findings, and Clinical Follow-up of a Systemic Lupus Erythematosus Case

“…In addition, a variety of neurological and psychiatric symptoms are frequently seen in SLE (3,4). According to the neuropsychiatric SLE criteria defined by Association of American Rheumatology (ACR), rate of ne uropsychiatric symptoms varies between 37% and 95% in SLE patients (5,6). These neuropsychiatric manifestations include delirium, anxiety disorders, cognitive disorders, mood disorders, and psychotic disorders (3,6).…”