Untangling the Web of Systemic Autoinflammatory Diseases

Author:

Rigante Donato1,Lopalco Giuseppe2,Vitale Antonio3,Lucherini Orso Maria3,Caso Francesco3,De Clemente Caterina3,Molinaro Francesco4,Messina Mario4,Costa Luisa5,Atteno Mariangela5,Laghi-Pasini Franco6,Lapadula Giovanni2,Galeazzi Mauro3,Iannone Florenzo2,Cantarini Luca3

Affiliation:

1. Institute of Pediatrics, Policlinico A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy

2. Interdisciplinary Department of Medicine, Rheumatology Unit, University of Bari, Bari, Italy

3. Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Viale Bracci 1, 53100 Siena, Italy

4. Division of Pediatric Surgery, Department of Medical Sciences, Surgery, and Neuroscience, University of Siena, Siena, Italy

5. Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy

6. Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy

Abstract

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-1βand 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.

Funder

Novartis

Publisher

Hindawi Limited

Subject

Cell Biology,Immunology

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