Neuropsychiatric Systemic Lupus Erythematosus: A 2021 Update on Diagnosis, Management, and Current Challenges

Abstract

Patients with systemic lupus erythematosus (SLE) experience neuropsychiatric symptoms. The term neuropsychiatric SLE (NPSLE) is a generic term that refers to a series of neurological and psychiatric symptoms directly related to SLE. In approximately 30% of patients with neuropsychiatric symptoms, SLE is the primary cause (NPSLE), and symptoms manifest more frequently around SLE onset. Neurovascular and psychotic conditions can also lead to NPSLE. Pathogenesis of NPSLE is implicated in both neuroinflammatory and ischemic mechanisms, and it is associated with high morbidity and mortality. After diagnosing and assigning causality, NPSLE treatment is individualized according to the type of neuropsychiatric manifestations, type of the predominant pathway, activity of SLE, and severity of the clinical manifestations. There are many problems to be addressed with regards to the diagnosis and management of NPSLE. Controlled clinical trials provide limited guidance for management, and observational cohort studies support symptomatic, antithrombotic, and immunosuppressive agents. The purpose of this review was to provide a detailed and critical review of the literature on the pathophysiology, diagnosis, and treatment of NPSLE. This study aimed to identify the shortcoming in diagnostic biomarkers, novel therapies against NPSLE, and additional research needs.

Case Report: Rapid Progression of Cognitive Dysfunction as an Initial Feature of Systemic Lupus Erythematosus With Leukoencephalopathy: A Case Report and Literature Review

“…Diagnosis of NPSLE is the lack of specific and sensitive CSF testing ( 6 ). As previous studies have shown, some autoantibodies have been suggested as a potential biomarker for diagnosis and therapeutic decision, such as antineuronal, anti-ribosomal P, and ant-NR2 antibodies ( 6 ).…”

“…Diagnosis of NPSLE is the lack of specific and sensitive CSF testing ( 6 ). As previous studies have shown, some autoantibodies have been suggested as a potential biomarker for diagnosis and therapeutic decision, such as antineuronal, anti-ribosomal P, and ant-NR2 antibodies ( 6 ). Autoantibodies may be detected in the CSF as a result of the transfer of peripherally produced autoantibodies across a breached blood brain barrier or increased intrathecal production ( 2 ).…”

“…Based on the relatively severe pathologic injury in brain, we might make an explanation on severe cognitive dysfunction in our patient. Diagnosis of NPSLE is the lack of specific and sensitive CSF testing (6). As previous studies have shown, some autoantibodies have been suggested as a potential biomarker for diagnosis and therapeutic decision, such as antineuronal, antiribosomal P, and ant-NR2 antibodies (6).…”

“…While neuropsychiatric events as initial symptoms in SLE patients are rare ( 4 ). Moreover, neuropsychiatric manifestations were classified into focal and diffuse neuropsychological syndromes ( 6 ). In the diffuse neuropsychological syndromes, isolated diffuse leukoencephalopathy with acute confusional state or cognitive dysfunction in lupus has rarely been reported and presents benign or malignant outcomes ( 7 ).…”

Fatigue have impact on the sexual problems in Chinese females with systemic lupus erythematosus

“…Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease that predominantly affects women between puberty and menopause [ 1 , 2 ]. SLE patients can suffer from clinical manifestations including arthritis, serositis, nephritis, rashes, scars, depigmentation, skin dimpling, hair loss, or neuropsychiatric problems [ 3 ], which have great impacts on quality of life (QoL) in this population [ 4 ]. Sexual problem is an important aspect of QoL and it is of great importance for SLE patients because it occurs predominantly in women with female to male ratio of 9:1 [ 5 ].…”