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Review
. 2022 Aug 24;11(17):4955.
doi: 10.3390/jcm11174955.

Progress in the Pathogenesis and Treatment of Neuropsychiatric Systemic Lupus Erythematosus

Minhui Wang  1 , Ziqian Wang  1 , Shangzhu Zhang  1 , Yang Wu  1 , Li Zhang  1 , Jiuliang Zhao  1 , Qian Wang  1 , Xinping Tian  1 , Mengtao Li  1 , Xiaofeng Zeng  1
Affiliations
Review

Progress in the Pathogenesis and Treatment of Neuropsychiatric Systemic Lupus Erythematosus

Minhui Wang et al. J Clin Med. .

Abstract

Neuropsychiatric systemic lupus erythematosus (NPSLE) has a broad spectrum of subtypes with diverse severities and prognoses. Ischemic and inflammatory mechanisms, including autoantibodies and cytokine-mediated pathological processes, are key components of the pathogenesis of NPSLE. Additional brain-intrinsic elements (such as the brain barrier and resident microglia) are also important facilitators of NPSLE. An improving understanding of NPSLE may provide further options for managing this disease. The attenuation of neuropsychiatric disease in mouse models demonstrates the potential for novel targeted therapies. Conventional therapeutic algorithms include symptomatic, anti-thrombotic, and immunosuppressive agents that are only supported by observational cohort studies, therefore performing controlled clinical trials to guide further management is essential and urgent. In this review, we aimed to present the latest pathogenetic mechanisms of NPSLE and discuss the progress in its management.

Keywords: management; neuropsychiatric lupus erythematosus; novel targeted therapies; pathogenesis; systemic lupus erythematosus.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Pathogenetic mechanisms in diffuse NPSLE.
Figure 2
Figure 2
Management for patients with neuropsychiatric systemic lupus erythematosus (NPSLE). Created with biorender.com.
See this image and copyright information in PMC

References

    1. Li M., Wang Y., Zhao J., Wang Q., Wang Z., Tian X., Zeng X. Chinese SLE Treatment and Research group (CSTAR) registry 2009–2019: Major clinical characteristics of Chinese patients with systemic lupus erythematosus. Rheumatol. Immunol. Res. 2021;2:43–47. doi: 10.2478/rir-2021-0001. - DOI - PMC - PubMed
    1. Wang Z., Li M., Ye Z., Li C., Li Z., Li X., Wu L., Liu S., Zuo X., Zhu P., et al. Long-term outcomes of patients with systemic lupus erythematosus: A Multicenter Cohort Study from CSTAR registry. Rheumatol. Immunol. Res. 2021;2:195–202. doi: 10.2478/rir-2021-0025. - DOI - PMC - PubMed
    1. Liang M.H., Corzillius M., Bae S.C., Lew R.A., Fortin P.R., Gordon C., Isenberg D., Alarcón G.S., Straaton K.V., Denburg J., et al. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999;42:599–608. doi: 10.1002/1529-0131(199904)42:43.0.Co;2-f. - DOI - PubMed
    1. Bortoluzzi A., Piga M., Silvagni E., Chessa E., Mathieu A., Govoni M. Peripheral nervous system involvement in systemic lupus erythematosus: A retrospective study on prevalence, associated factors and outcome. Lupus. 2019;28:465–474. doi: 10.1177/0961203319828499. - DOI - PubMed
    1. Hanly J.G., Li Q., Su L., Urowitz M.B., Gordon C., Bae S.C., Romero-Diaz J., Sanchez-Guerrero J., Bernatsky S., Clarke A.E., et al. Peripheral Nervous System Disease in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study. Arthritis Rheumatol. 2020;72:67–77. doi: 10.1002/art.41070. - DOI - PMC - PubMed

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