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Review
. 2021 Sep 14;13(9):e17969.
doi: 10.7759/cureus.17969. eCollection 2021 Sep.

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

Sobia Sarwar  1 , Alaa S Mohamed  2 , Sylvette Rogers  3 , Shah T Sarmast  4 , Saurabh Kataria  5   6   7 , Khalid H Mohamed  8 , Muhammad Zain Khalid  9 , Mohammad Omar Saeeduddin  10 , Saher T Shiza  11 , Sarfaraz Ahmad  12 , Anum Awais  13 , Romil Singh  14
Affiliations
Review

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

Sobia Sarwar et al. Cureus. .

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.

Keywords: clinical presentation; cns lupus; diagnosis; management; neuropsychiatric sle; pathogenesis.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Factors involved in the pathogenesis of neuropsychiatric SLE
TNF: tumor necrosis factor, HLA: human leukocyte antigen, IL: interleukin, GABA: gamma-aminobutyric acid, UCH-L1: ubiquitin carboxyl-terminal hydrolase isozyme L1, RNP: ribonucleoprotein, IFN: interferon, aCL: anticardiolipin, GFAP: glial fibrillary acid protein, APRIL: a proliferation-inducing protein, IP: interferon-gamma induced protein, CCL: chemokine ligand, PAI: plasminogen activator inhibitor, LAC: lupus anticoagulant.
Figure 2
Figure 2. Pathogenic pathways for neuropsychiatric SLE
BBB: Blood-brain barrier, aPL: Antiphospholipid autoantibodies.
Figure 3
Figure 3. Treatment options in neuropsychiatric SLE
BBB: Blood-brain barrier, NPSLE: Neuropsychiatric systemic lupus erythematosus, aPL: Antiphospholipid autoantibodies.
See this image and copyright information in PMC

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