Autoinflammatory diseases - a new entity of inflammation

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Author(s)

    • Heike Toshio
    • Department of Physiology, Keio University School of Medicine
    • Saito Megumu K
    • Clinical Application Department, Center for iPS cell research and application, Kyoto University
    • Nakahata Tatsutoshi
    • Clinical Application Department, Center for iPS cell research and application, Kyoto University

Abstract

The autoinflammatory diseases are characterized by seemingly unprovoked episodes of inflammation, without high-titer autoantibodies or antigen-specific T cells. The concept was proposed ten years ago with the identification of the genes underlying hereditary periodic fever syndromes. NLRP3 inflammasome activation and IL-1β secretion have recently emerged as a central mechanism in the pathogenesis of disease. Here we describe four genetically defined syndromes like cryopyrin-associated periodic syndromes (CAPS, cryopyrinopathies), mevalonate kinase deficiency (MKD) or hyper-IgD and periodic fever syndrome (HIDS), pyogenic aseptic arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome), and deficiency of interleukin-1-receptor antagonist (DIRA) along with the pitfall for understanding the pathphysiology.

Journal

  • Inflammation and Regeneration

    Inflammation and Regeneration 31(2), 125-136, 2011

    The Japanese Society of Inflammation and Regeneration

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