Epidemiology, characterization, and diagnosis of neuropsychiatric events in systemic lupus erythematosus

ABSTRACT

Introduction: Neuropsychiatric systemic lupus erythematosus (NPSLE) is characterized by a heterogeneity of clinical manifestations. The absence of diagnostic criteria and the lack of clinical trials is a challenge in clinical practice.

Areas covered: A literature review was performed to describe epidemiology, characterization (clinical, immunological, and imaging), diagnosis and treatment of NPSLE. Classification criteria have been the first step towards a uniform definition. More recently, different attribution models have been developed to help to determine if the NP event is due to SLE. Disease activity is a major risk factor for NP events. Cytokines and autoantibodies are associated with NP events, however, only a few studies have identified risk factors for individual NP events.

Expert opinion: Further research needs to search for and validate biomarkers for NPSLE and individual NP events, including neuroimaging findings, attribution models, and serologic markers. This will be a fundamental step in planning randomized control trials in the treatment of NPSLE to improve outcome.

KEYWORDS:

• Neuropsychiatric SLE
• systemic lupus erythematosus
• treatment
• clinical characterization
• diagnosis of NPSLE

Article highlights

• NPSLE is still a challenge in clinical practice and research settings.

• Conventional MRI techniques lack specificity, multimodalities, functional MRI sequences can identify reversible structural and functional brain features.

• Disease activity is a major risk factor for NP events.

• Autoantibodies such as aPL have been associated with ischemic cerebrovascular manifestations and non-ischemic manifestations such as seizures, headache, and cognitive impairment.

• Diffuse NP manifestations have been associated with the presence of anti-ribosomal P antibodies and NR2 antibodies.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This work is funded by Brazilian National Council for Scientific and Technological Development (CNPq) (157534/2015-4 to SA; scholarship to JCA) and Coordenação de Aperfeiçoamento de Pessoal de Nível Superior - Brasil (CAPES) (Finance Code 001).