Working the endless puzzle of hereditary autoinflammatory disorders. Issue 3 (May 2014)

• Record Type: Journal Article
• Authors: Caso, Francesco; Cantarini, Luca; Lucherini, Orso Maria; Sfriso, Paolo; Fioretti, Maria; Costa, Luisa; Vitale, Antonio; Atteno, Mariangela; Galeazzi, Mauro; Muscari, Isabella; Magnotti, Flora; Frediani, Bruno; Punzi, Leonardo; Rigante, Donato
• Is Part Of: Modern rheumatology. Volume 24:Issue 3(2014)
• Page Start: 381
• Page End: 389
• Publication Date: 2014-05
• Subjects: Rheumatology -- Periodicals; 616.723005
• DOI: 10.3109/14397595.2013.843755
• Languages: English
• ISSNs: 1439-7595

Abstract

<abstract> <title>Abstract</title> <p>Hereditary autoinflammatory disorders encompass manifold dysfunctions of innate immunity caused by mutations in genes coding for the main characters of the inflammatory scene: most of these conditions have an early onset, ranging from the first days of life to the first decades, and include hereditary periodic fevers, NLRP-related diseases, granulomatous and pyogenic syndromes, which are basically characterized by upturned inflammasome activity and overproduction of bioactive interleukin (IL)-1β and other proinflammatory cytokines. The discovery of a causative link between autoinflammation and IL-1β release has improved our understanding of the intimate mechanisms of innate immunity, and has likewise led to the identification of extraordinary treatments for many of these disorders.</p> </abstract>