Neuropsychiatric Systemic Lupus Erythematosus Involvement: Towards a Tailored Approach to Our Patients?

Abstract

Neuropsychiatric involvement in systemic lupus erythematosus (NPSLE) is a complex condition that remains poorly understood, and includes heterogeneous manifestations involving both the central and peripheral nervous system, with disabling effects. There are several models to improve NPSLE diagnosis when a neurological syndrome is present. In the last couple of years, the growing knowledge of the role of cytokines and antibodies in NPSLE, as well as the development of new functional imaging techniques, has brought some insights into the physiopathology of the disease, but their validation for clinical use remains undetermined. Furthermore, besides the classic clinical approach, a new tool for screening the 19 NPSLE syndromes has also been developed. Regarding NPSLE therapeutics, there is still no evidence-based treatment approach, but some data support the safety of biological medication when classic treatment fails. Despite the tendency to reclassify SLE patients in clinical and immunological subsets, we hope that these data will inspire medical professionals to approach NPSLE in a manner more tailored to the individual patient.

Primary Psychiatric Disorder Masking the Diagnosis of Neuropsychiatric Lupus in a Patient with Altered Mental Status: A Case Report

“…Interestingly, the initial value was 87.6%, however, it dropped significantly after the exclusion of patients with characteristics that would make the diagnosis of NPSLE less likely. These included (a) neuropsychiatric event taking place more than six months before the diagnosis of SLE, (b) SLE cofounders more likely to cause the event, and (c) meeting the “Ainala Criteria” (headaches, mild depression, anxiety, minor cognitive complaints, and electromyography-negative polyneuropathy) [ 3 ].…”

“…In 1999, the American College of Rheumatology made recommendations regarding NPSLE based on 19 neuropsychiatric conditions, including 12 from central nervous system (CNS) and seven from the peripheral nervous system (PNS) [ 3 ]. The CNS conditions include, but are not limited to, headache, seizure disorder, cerebrovascular disease, cognitive dysfunction, mood disorder, anxiety disorder, psychosis, and acute confusional state.…”

“…These antibodies specifically target the hippocampus and amygdala, which can explain their association with depression and cognitive dysfunction. Anti-dsDNA antibodies, well known to be involved in the SLE disease inflammatory process, have been shown to recognize a specific sequence in the N-methyl-D-aspartate receptors NR2a and NR2b [ 3 ]. The anti-dsDNA and anti-NR2 antibodies are detected in serum and CSF in approximately 25-50% of SLE patients and there have been associations between their levels and NPSLE symptoms, specifically mood disorders, acute confusional state, and cognitive decline.…”

Rituximab as First-Line Therapy in Severe Lupus Erythematosus with Neuropsychiatric and Renal Involvement: A Case-Report and Review of the Literature

“…In contrast to other systemic manifestations of SLE, the prominent feature of NPSLE is not the presence of vasculitis [ 18 ]. Instead, growing evidence shows that blood–brain barrier (BBB) dysfunction is essential to the development of NPSLE, allowing the passive diffusion of auto-reactive antibodies and cytokines, facilitating the development of a pro-inflammatory features [ 19 , 20 ].…”

The introspection on the diagnosis and treatment process of a case of Guillain–Barré syndrome (GBS) attributed to systemic lupus erythematosus (SLE)

“…[ 18 ] NPSLE is typically diagnosed when the patient is symptomatic (based on patient complaints or the physician's assessment). [ 19 ] Due to the lack of a gold standard, the attribution of NPSLE represents a clinical challenge; diagnosis is largely based on exclusion of other potential causes. NP events in patients with SLE have a detrimental effect on the quality of life and are associated with a poor prognosis.…”