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Autoinflammatory Diseases

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Abstract

Autoinflammatory diseases (AIDs) are a group of systemic illnesses that cause unprovoked episodes of inflammation resulting from abnormalities of the innate immune system. Since 1997, when mutations in the Mediterranean fever (MEFV) gene were found to cause familial Mediterranean fever, more than three dozen monogenic AIDs have been identified. Increased recognition of these illnesses has provided a better understanding of their diverse clinical presentation, including how various AIDs affect the central and peripheral nervous systems.

More recently, diseases such as Aicardi-Goutières syndrome, which induce excessive type I interferon production, have been reclassified as autoinflammatory because of the activation of the innate immune system in the absence of an external trigger. These diseases are now being referred to as “interferonopathies” and will also be addressed within this chapter. An improved understanding of the neurological manifestations of AIDs will help the clinician recognize and treat these rare illnesses.

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Authors and Affiliations

  1. Autoinflammatory Diseases Clinic, Program in Rheumatology, Boston Children’s Hospital, Boston, MA, USA

    Jonathan S. Hausmann & Fatma Dedeoglu

  2. Autoinflammatory Diseases Clinic, Department of Rheumatology, Beth Israel Deaconess Medical Center, Boston, MA, USA

    Jonathan S. Hausmann

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  1. Jonathan S. Hausmann
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  2. Fatma Dedeoglu
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Correspondence to Jonathan S. Hausmann .

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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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Hausmann, J.S., Dedeoglu, F. (2019). Autoinflammatory Diseases. In: Cho, T., Bhattacharyya, S., Helfgott, S. (eds) Neurorheumatology. Springer, Cham. https://doi.org/10.1007/978-3-030-16928-2_13

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  • DOI: https://doi.org/10.1007/978-3-030-16928-2_13

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