Article Text

other Versions

Download PDFPDF
Autoinflammatory syndromes in neurology: when our first line of defence misbehaves
  1. William K Diprose1,2,
  2. Anthony Jordan3,
  3. Neil E Anderson1
  1. 1Department of Neurology, Auckland City Hospital, Auckland, New Zealand
  2. 2Department of Medicine, Faculty of Medical and Health Sciences, The University of Auckland, Auckland, New Zealand
  3. 3Department of Clinical Immunology and Allergy, Auckland City Hospital, Auckland, New Zealand
  1. Correspondence to Dr William K Diprose, Department of Neurology, Auckland City Hospital, Auckland 1023, New Zealand; william.diprose{at}


Autoinflammatory syndromes result in a defective innate immune system. They are characterised by unexplained fever and systemic inflammation involving the skin, muscle, joints, serosa and eyes, along with elevated acute phase reactants. Autoinflammatory syndromes are increasingly recognised as a cause of neurological disease with a diverse range of manifestations. Corticosteroids, colchicine and targeted therapies are effective if started early, and hence the importance of recognising these syndromes. Here, we review the neurological features of specific autoinflammatory syndromes and our approach (as adult neurologists) to their diagnosis.

  • neuroimmunology
  • immunology
  • rheumatology
  • clinical neurology
  • haematology

Statistics from


  • Contributors WKD was commissioned to write the article. AJ provided immunology expertise. NEA provided neuroimmunology expertise. All authors contributed to writing and approving the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned. Externally peer reviewed by Neil Scolding, Bristol, UK.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Linked Articles

  • Editors’ commentary
    Phil E M Smith Geraint N Fuller

Other content recommended for you