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Mediators of Inflammation
Volume 2014, Article ID 948154, 15 pages
Review Article

Untangling the Web of Systemic Autoinflammatory Diseases

1Institute of Pediatrics, Policlinico A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy
2Interdisciplinary Department of Medicine, Rheumatology Unit, University of Bari, Bari, Italy
3Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Viale Bracci 1, 53100 Siena, Italy
4Division of Pediatric Surgery, Department of Medical Sciences, Surgery, and Neuroscience, University of Siena, Siena, Italy
5Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy
6Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy

Received 6 March 2014; Revised 28 April 2014; Accepted 29 April 2014; Published 15 July 2014

Academic Editor: Fulvio D’Acquisto

Copyright © 2014 Donato Rigante et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


The innate immune system is involved in the pathophysiology of systemic autoinflammatory diseases (SAIDs), an enlarging group of disorders caused by dysregulated production of proinflammatory cytokines, such as interleukin-1β and tumor necrosis factor-α, in which autoreactive T-lymphocytes and autoantibodies are indeed absent. A widely deranged innate immunity leads to overactivity of proinflammatory cytokines and subsequent multisite inflammatory symptoms depicting various conditions, such as hereditary periodic fevers, granulomatous disorders, and pyogenic diseases, collectively described in this review. Further research should enhance our understanding of the genetics behind SAIDs, unearth triggers of inflammatory attacks, and result in improvement for their diagnosis and treatment.