Abstract
Neuropsychiatric (NP) involvement in systemic lupus erythematosus (SLE) is one of the most severe manifestations of the disease that has a heavy impact on patient’s functioning, quality of life, and disease outcome. The prevalence is highly variable and the clinical phenotypes vary from common syndromes to rare NP entities. Its occurrence may be the result of a primary manifestation of SLE, secondary to other conditions (such as infections or metabolic disturbances) or the effect of concomitant comorbidities that often complicate the disease course. Correct attribution of NP events may pose diagnostic challenges and it is a critical factor in selecting the correct management. Although there is still no diagnostic gold standard to rightly diagnose NPSLE syndromes, great advances have been made in improving the clinician judgment in the evaluation process. In this narrative review, we present and discuss available evidence concerning NPSLE with a special focus on the attribution models developed using composite decision rules to ascribe NP events to SLE.
Highlights
Neuropsychiatric (NP) involvement in systemic lupus erythematosus (SLE) remains one of the most challenging manifestations of the disease
One of the major issues that makes the epidemiology of NPSLE so poorly defined is the “attribution” of NP event, a process aimed at establishing whether a physiopathologic link exists between a given NP event and the underlying disease [2]
Another important landmark was represented by the 1999 American College of Rheumatology (ACR) nomenclature which, up to now, is still the reference for the assessment of NP manifestations occurring in SLE patients [4]
Summary
Department of Medical Sciences, Section of Rheumatology, University of Ferrara, Azienda Ospedaliero-Universitaria Sant’Anna di Ferrara, Cona, Italy Attikon, Greece Nicolai Leuchten, Universitätsklinikum Carl Gustav Carus, Germany Anabela Barcelos, Centro Hospitalar Baixo Specialty section: This article was submitted to Rheumatology, a section of the journal
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