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Autoinflammatory Disorders

  • Chapter
Book cover Primary Immunodeficiency Diseases

  • Autoinflammatory disorders constitutes a new term that covers a group of diseases characterized by recurrent generalized inflammation in the absence of infectious or autoimmune causes.

  • Dysregulation of the innate immune system causes inflammation by affecting the pathways connected to the NOD-like receptors.

  • The patients are at risk of developing amyloidosis, the main long-term sequelae of many of these disorders.

  • The term was initially used only for the inherited periodic fever syndromes.

  • Over the past few years, the concept of autoinflammatory disorders has expanded to encompass an increasing number of polygenic/multifactorial diseases.

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  1. Department of Pediatrics, Göteborg University The Queen Silvia Children’s Hospital, SE-416 85, Göteborg, Sweden

    Stefan Berg MD, PhD & Anders Fasth MD, PhD

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  1. Stefan Berg MD, PhD
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  1. Immunology, Asthma and Allergy Research Institute Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, 14194, Iran

    Nima Rezaei

  2. Section of Infection, Inflammation and Immunity School of Medicine and Biomedical Sciences, The University of Sheffield, S10 2RX, Sheffield, UK

    Nima Rezaei

  3. Division of Allergy and Clinical Immunology Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, 14194, Iran

    Asghar Aghamohammadi

  4. Division of Immunology Children’s Hospital, Harvard Medical School, Boston, MA, 02115, USA

    Luigi D. Notarangelo

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Berg, S., Fasth, A. (2008). Autoinflammatory Disorders. In: Rezaei, N., Aghamohammadi, A., Notarangelo, L.D. (eds) Primary Immunodeficiency Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-78936-9_7

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