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Review Article

Neuropsychiatric Systemic Lupus Erythematosus: A Remaining Challenge

Author(s): Daniel Mrak, Michael Bonelli* and Helga Radner

Volume 28, Issue 11, 2022

Published on: 27 May, 2022

Page: [881 - 891] Pages: 11

DOI: 10.2174/1381612828666220512102824

Price: $65

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Abstract

Systemic Lupus Erythematosus (SLE) is an autoimmune disease, which affects a wide range of organs with variable clinical features. Involvement of the nervous system is a challenging and multifaceted manifestation of the disease, presenting with a broad range of symptoms. Neuropsychiatric lupus (NPSLE) encompasses seven syndromes of the peripheral and 12 of the central nervous system, associated with a high disease burden. Despite advances in the management of SLE, NP manifestations still pose a challenge to clinicians. First, diagnosis and attribution of SLE are difficult due to the lack of specific biomarkers or imaging modalities. Second, therapeutic options are limited, and evidence is mainly based on case reports and expert consensus, as clinical trials are sparse. Moreover, no validated outcome measure on disease activity exists. Current recommendations for treatment include supportive as well as immunosuppressive medication, depending on the type and severity of manifestations. As NPSLE manifestations are increasingly recognized, a broader spectrum of therapeutic options can be expected.

Keywords: Central nervous system, peripheral nervous system, neuropsychiatric systemic lupus erythematosus, systemic lupus erythematosus, autoimmune disease, constitutional symptoms.

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