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

Sobia Sarwar,Romil Singh,Alaa S Mohamed,Sarfaraz Ahmad,Shah T Sarmast,Mohammad Omar Saeeduddin,Saurabh Kataria,Anum Awais,Saher T Shiza,Muhammad Zain Khalid,Khalid H Mohamed,Sylvette Rogers

doi:10.7759/cureus.17969

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.

Highlights

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by the involvement of almost every organ of the body, a broad spectrum of clinical manifestations, and several immunemediated abnormalities leading to multiple organ dysfunction [1]
There is no potential agent against neuropsychiatric SLE (NPSLE)
Evaluating and designing effective interventions requires an understanding of the pathophysiology that led to NPSLE

Summary

Introduction

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by the involvement of almost every organ of the body, a broad spectrum of clinical manifestations, and several immunemediated abnormalities leading to multiple organ dysfunction [1]. Antibody level increases in SLE and NPSLE and is associated with generalized disease activity, cognitive dysfunction, and psychiatric manifestations. By combining the clinical evaluation, serological studies, and imaging results, we can proclaim that patient has active NPSLE or having symptoms due to other causes [5]. They highlighted the sensitivity and specificity of positive antibody tests in NPSLE 84% and 53%, respectively, with a positive predictive value of 71.1% He reported that the SLE disease activity index score and positive skin manifestation might be helpful in the diagnosis of NPSLE [14]. The European League Against Rheumatism (EULAR) published a consensus and gave possible recommendations for NPSLE management These recommendations include a general therapeutic approach that does not vary from non-SLE patients presenting with neuropsychiatric manifestations. New clinical trials should evaluate the potential options such as neuroimaging, validating outcome measures, and therapy for non-emergent neuropsychiatric events, including mood disorders [36,37]

Conclusions

Findings

Disclosures