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Behçet’s syndrome pathophysiology and potential therapeutic targets

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Abstract

Behçet syndrome is a systemic inflammatory disorder characterized by multiorgan involvement such as oral and genital ulcers, uveitis, skin lesions as well as by less frequent, but often more severe, central nervous system and vascular manifestations. The pathogenetic mechanisms are still incompletely known; however the interaction between a specific genetic background and environmental or infectious factors certainly contributes to the immune dysregulation that characterizes this disease. The discovery of new immunological pathways in Behçet syndrome pathogenesis may help us to set up new treatments. In this review, we will focus our attention on the possible mechanisms underlying Behçet syndrome pathogenesis and their potential role as novel therapeutic targets.

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Acknowledgments

The authors wish to thank the Associazione Italiana Sindrome e Malattia di Behçet (SIMBA) for its support to their studies.

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Authors and Affiliations

  1. SOD Patologia Medica, Center for Autoimmune Systemic Diseases, Behçet Center and Lupus Clinic, AOU Careggi, Florence, Italy

    Elena Silvestri, Danilo Squatrito, Mario Milco D’Elios, Domenico Prisco & Lorenzo Emmi

  2. Department of Experimental and Clinical Medicine, University of Florence, L.go G. Brambilla 3, 50134, Florence, Italy

    Giacomo Emmi, Elena Silvestri, Mario Milco D’Elios, Lucia Ciucciarelli & Domenico Prisco

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  1. Giacomo Emmi
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  2. Elena Silvestri
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  3. Danilo Squatrito
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  4. Mario Milco D’Elios
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  5. Lucia Ciucciarelli
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  7. Lorenzo Emmi
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Correspondence to Giacomo Emmi.

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Emmi, G., Silvestri, E., Squatrito, D. et al. Behçet’s syndrome pathophysiology and potential therapeutic targets. Intern Emerg Med 9, 257–265 (2014). https://doi.org/10.1007/s11739-013-1036-5

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