TY - CHAP M1 - Book, Section TI - Chapter 209. Autoinflammatory Disorders A1 - Hashkes, Philip J. A2 - Rudolph, Colin D. A2 - Rudolph, Abraham M. A2 - Lister, George E. A2 - First, Lewis R. A2 - Gershon, Anne A. PY - 2011 T2 - Rudolph's Pediatrics, 22e AB - 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. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/18 UR - accesspediatrics.mhmedical.com/content.aspx?aid=7019999 ER -