ReviewCentral nervous system involvement in systemic lupus erythematosus: Overview on classification criteria
Introduction
Nervous system involvement has been recognised in systemic lupus erythematosus (SLE) patients for over 100 years and several classification criteria have been proposed in an attempt to capture the diverse clinical expressions. Since the first description in the 19th century by Kaposi and Osler of an SLE patient with pleurisy, pneumonia, disturbed neurologic function, and rapid progression to death [1], [2], it soon became obvious that neuropsychiatric manifestations in SLE are frequent and broad and that management is not an easy task. Neuropsychiatric SLE includes a variety of focal, diffuse, central, peripheral, psychiatric, isolated, complex, simultaneous and sequential symptoms and signs, representing both active and inactive disease states. Central nervous system disease predominates and may take the form of either diffuse disease (e.g. psychosis or depression) or focal disease (e.g. stroke or transverse myelitis) [3], [4], [5], [6], [7].
In this Review, we sought to summarise the current insights on the various aspects of neuropsychiatric SLE with special emphasis on the terminology and classification criteria needed to correctly attribute the particular event to SLE.
Section snippets
Search strategy and selection criteria
References were selected by a PubMed search of English language publications and covered the period from 1946 to April 2012. Search terms included “systemic lupus erythematosus”, “lupus”, “neuropsychiatric”, “neuropsychiatric lupus classification”, “neuropsychiatric lupus criteria”, “neuro-lupus”, and “cerebrovascular lupus”. Further articles were identified through the references cited in those articles. Abstracts were reviewed, and when relevant findings were reported, the full article was
American College of Rheumatology (ACR) classification criteria for SLE and neuropsychiatric involvement
The first preliminary classification criteria for SLE dates back to 1971 when seizures, psychosis and focal neurologic disorders were the only neuropsychiatric manifestations encompassed [8]. The succeeding 1982 revised criteria considered dementia and coma among the NP clinical settings to be evaluated. Nevertheless, only seizures and psychosis were formally included in this revised classification criteria [9], which is far from enough to cover the whole spectrum of neuropsychiatric
ACR case definitions for neuropsychiatric SLE
An ad hoc multidisciplinary committee of 35 members representing the areas of rheumatology, neurology, psychiatry, neuro-psychiatry and haematology was convened by the American College of Rheumatology Research Committee in April of 1997 for the purpose of developing standard nomenclature for neuropsychiatric SLE [13]. The committee developed neuropsychiatric SLE case definitions with diagnostic criteria, exclusions, associations and ascertainment. Standards and recommendations were also
Current ACR classification criteria: grey areas and potential solutions
Several studies have criticised the ACR nomenclature [22] and some of them have proposed revised versions for the classification, claiming that the proposed 19 ACR criteria did not differentiate SLE patients from controls, nor neuropsychiatric SLE patients from other SLE patients [15], [22], [23], [24], [25], [26]. Some authors have reported an inadequate performance of the proposed ACR criteria, criticising the lack of criteria based on neuro-pathogenic features [15].
As a matter of fact, some
Take-home messages
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Central nervous system involvement is one of the major causes of morbidity and mortality in systemic lupus erythematosus (SLE) patients.
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Neuropsychiatric SLE includes a variety of focal, diffuse, central, peripheral, psychiatric, isolated, complex, simultaneous and sequential symptoms and signs, representing both active and inactive disease states.
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The American College of Rheumatology case definition represents a step forward in the diagnosis and management of SLE patients; in spite of this, some
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2017, Autoimmunity ReviewsCitation Excerpt :In 1999 the American College of Rheumatology (ACR) defined 19 neuropsychiatric syndromes in SLE from which 12 involve the central nervous system (CNS-SLE) and 7 the peripheral nervous system (PNS-SLE) [5]. In the last decade, CNS-SLE involvement has been the focus of numerous studies, thus allowing to its clinical characterization, association with immunological markers or their relationship with SLE activity [6–11]. In contrast, PNS-SLE has been scarcely studied [12–15], despite its association with significant morbidity and a worsen quality of life [16].