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Behçet syndrome: from pathogenesis to novel therapies

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Abstract

Behçet syndrome is a chronic disease hallmarked by inflammation of the blood vessels that is related to an autoimmune reaction caused by inherited susceptibility due to specific genes and environmental factors, probably components of infectious microorganisms, which turn on or get going the disease in genetically susceptible subjects. The more common clinical expression of the disease is represented by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis, sometimes associated with inflammatory arthritis, phlebitis, iritis, as well as inflammation of the digestive tract, brain, and spinal cord. The treatment strategies used to manage the manifestations of Behçet syndrome have gradually progressed, and a number of new therapeutic resources have been implemented in recent years, allowing better control of pathogenic mechanisms, reducing symptoms and suffering, and ameliorating patient’s outcome.

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Acknowledgments

The study was supported by the “5 × 1,000” voluntary contribution and by a Grant (GM) from the Italian Ministry of Health (RC1201ME04, RC1203ME46, RC1302ME31, and RC1403ME50) through Department of Medical Sciences, Division of Internal Medicine and Chronobiology Unit, IRCCS Scientific Institute and Regional General Hospital “Casa Sollievo della Sofferenza”, Opera di Padre Pio da Pietrelcina, San Giovanni Rotondo (FG), Italy.

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The authors declare that there are no conflicts of interest with respect to the authorship and/or publication of this article.

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  1. Division of Internal Medicine and Chronobiology Unit, Department of Medical Sciences, IRCCS Scientific Institute and Regional General Hospital “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, Italy

    Gianluigi Mazzoccoli & Rosa Rubino

  2. Division of Internal Medicine and Rheumatology Unit, Department of Medical Sciences, IRCCS Scientific Institute and Regional General Hospital “Casa Sollievo della Sofferenza”, San Giovanni Rotondo, FG, Italy

    Angela Matarangolo, Michele Inglese & Angelo De Cata

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  1. Gianluigi Mazzoccoli
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Correspondence to Gianluigi Mazzoccoli or Angelo De Cata.

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Mazzoccoli, G., Matarangolo, A., Rubino, R. et al. Behçet syndrome: from pathogenesis to novel therapies. Clin Exp Med 16, 1–12 (2016). https://doi.org/10.1007/s10238-014-0328-z

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