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Central Nervous System Vasculitis

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Neurorheumatology

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Abstract

Central nervous system (CNS) vasculitis is a heterogeneous group of diseases that has a common feature of inflammation of blood vessels in the brain, spinal cord, or meninges. Primary angiitis of the CNS (PACNS) is a single organ vasculitis where vasculitis is confined only to the CNS without other identified etiologies, whereas secondary CNS vasculitis occurs in the setting of other diseases that are known to be associated with CNS vasculitis. A thorough clinical examination, laboratory workup, and radiographic assessment are mandatory to evaluate secondary etiology and mimics of CNS vasculitis. Cerebrospinal fluid analysis is an essential part of the evaluation. Brain biopsy remains the gold standard for the diagnosis of CNS vasculitis; however, this may not be achievable or diagnostic due to various reasons. The treatment for PACNS is extrapolated from the treatment strategy of systemic small vessel vasculitides, and the data to prove efficacy for this strategy is scarce.

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Author information

Authors and Affiliations

  1. Division of Vasculitis, Immuno-Rheumatology Center, St. Luke’s International Hospital, Tokyo, Japan

    Hiromichi Tamaki

  2. Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Orthopedic and Rheumatologic Institute, Cleveland Clinic, Cleveland, OH, USA

    Rula A. Hajj-Ali

Authors
  1. Hiromichi Tamaki
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  2. Rula A. Hajj-Ali
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Correspondence to Rula A. Hajj-Ali .

Editor information

Editors and Affiliations

  1. Division of Neuroimmunology, University of Iowa, Iowa City, IA, USA

    Tracey A. Cho

  2. Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Shamik Bhattacharyya

  3. Division of Rheumatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Simon Helfgott

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Tamaki, H., Hajj-Ali, R.A. (2019). Central Nervous System Vasculitis. In: Cho, T., Bhattacharyya, S., Helfgott, S. (eds) Neurorheumatology. Springer, Cham. https://doi.org/10.1007/978-3-030-16928-2_12

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  • DOI: https://doi.org/10.1007/978-3-030-16928-2_12

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-16927-5

  • Online ISBN: 978-3-030-16928-2

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