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Autoinflammatory syndromes, formerly known as periodic fever syndromes, are defined as recurrent attacks of often unprovoked systemic inflammation that are related to a lack of adequate regulation of the innate immune system.1-3 Unlike autoimmune diseases, these conditions are not generally marked by autoantibodies or autoreactive T cells. Many of these syndromes have a genetic etiology3,4 (eTable 209.1) involving the excessive production and activity of interleukin (IL)-1β.5 The conditions are no longer known as periodic fever syndromes, 6-8 because most are not truly periodic nor is fever a necessary feature.

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eTable 209.1. Genetic Characteristics of the Inherited Autoinflammatory Syndromes
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As understanding of the pathogenesis of autoinflammatory syndromes increases, so does the spectrum of conditions that may be included within this category. Thus, systemic juvenile idiopathic arthritis, genetic inflammatory granulomatous diseases (early onset sarcoidosis and Blau syndrome) and Behçet disease (eTable 209.2) are now considered to be autoinflammatory disorders, although this chapter will be limited to conditions that do not fall into other diagnostic categories.

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eTable 209.2. The Risk of Amyloidosis in Untreated Autoinflammatory Syndromes
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These syndromes should be suspected in patients, mainly young children, with recurrent fever unexplained by infections and/or with episodic symptoms in various systems, especially the skin, gastrointestinal tract, joints, and eyes. A complete history and physical examination are crucial, and often careful determination of the organ systems involved, age of onset, length of attacks, intervals between attacks, and triggering events will allow the correct diagnosis to be suspected ...

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