Clinical Features

  • Yoshiyuki Arinuma
  • Shunsei Hirohata


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.


NPSLE ACR nomenclature Diffuse psychiatric/neuropsychological Syndromes Acute confusional state Neurologic syndromes 

Supplementary material

451310_1_En_5_MOESM1_ESM.avi (6.1 mb)
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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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Yoshiyuki Arinuma
    • 1
  • Shunsei Hirohata
    • 2
    • 3
  1. 1.Department of Rheumatology and Infectious diseasesKitasato University School of MedicineSagamiharaJapan
  2. 2.Department of RheumatologyNobuhara HospitalTatsunoJapan
  3. 3.Department of Rheumatology & Infectious DiseasesKitasato University School of MedicineSagamiharaJapan

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