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Clinical Features

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Neuropsychiatric Systemic Lupus Erythematosus

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Abstract

Clinical features in neuropsychiatric systemic lupus erythematosus (NPSLE) include a variety of manifestations, which are too complicated to understand. In 1999, the American college of rheumatology (ACR) proposed nomenclature and case definitions of NPSLE as nosology for clinical descriptions and research. Clinically, NPSLE is classified into 2 different categories; one is diffuse psychiatric/neuropsychological syndromes and the other is neurologic syndromes. This discrimination is not always advantageous for clinical practice including a diagnosis and therapeutic intervention. Moreover, the severities of each manifestation differ among NPSLE. For example, acute confusional state is very severe form with poor prognosis, whereas headache, mood disorders, anxiety disorders and cognitive dysfunction are sometimes mild, and are also common in individuals without SLE. Also, we need to remember that NPSLE can be developed even in the absence of systemic disease activities. Finally, some neuropsychiatric manifestations have not been adequately defined in the ACR nomenclature.

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Authors and Affiliations

  1. Department of Rheumatology and Infectious diseases, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan

    Yoshiyuki Arinuma

  2. Department of Rheumatology, Nobuhara Hospital, Tatsuno, Hyogo, Japan

    Shunsei Hirohata

  3. Department of Rheumatology & Infectious Diseases, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan

    Shunsei Hirohata

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  1. Yoshiyuki Arinuma
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Editors and Affiliations

  1. Department of Rheumatology, Nobubara Hospital, Tatsuno, Hyogo, Japan

    Shunsei Hirohata

5.1 Electronic Supplementary Material

Video 5.1

An SLE patient with acute confusional state. A 32-year-old female patient fell into a coma soon after admission. She kept opening her mouth with flattering her tongue, considered to be oral myoclonus (AVI 6254 kb)

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Arinuma, Y., Hirohata, S. (2018). Clinical Features. In: Hirohata, S. (eds) Neuropsychiatric Systemic Lupus Erythematosus. Springer, Cham. https://doi.org/10.1007/978-3-319-76496-2_5

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