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Mediators of Inflammation
Volume 2014, Article ID 107421, 14 pages
Review Article

Biological Treatments in Behçet’s Disease: Beyond Anti-TNF Therapy

1Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Viale Bracci 1, 53100 Siena, Italy
2Rheumatology Unit, Department of Medicine DIMED, University of Padova, Via Giustiniani 2, 35128 Padova, Italy
3Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Via S. Pansini 5, 80131 Naples, Italy
4Institute of Pediatrics, Cattolica Sacro Cuore University, Largo Agostino Gemelli 8, 00168 Rome, Italy
5La Sapienza University, Viale del Policlinico 155, 00161 Rome, Italy
6Department of Pediatrics, Rheumatology Unit, Anna Meyer Children’s Hospital and University of Florence, Viale Pieraccini 24, 50139 Florence, Italy
7Rheumatology Service, Hospital Regional Universitario Carlos Haya, University of Màlaga, Avenida Carlos Haya s/n, 29010 Màlaga, Spain

Received 16 January 2014; Revised 17 April 2014; Accepted 1 May 2014; Published 30 June 2014

Academic Editor: Chiara De Luca

Copyright © 2014 Francesco Caso 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.


Behçet’s disease (BD) is universally recognized as a multisystemic inflammatory disease of unknown etiology with chronic course and unpredictable exacerbations: its clinical spectrum varies from pure vasculitic manifestations with thrombotic complications to protean inflammatory involvement of multiple organs and tissues. Treatment has been revolutionized by the progressed knowledge in the pathogenetic mechanisms of BD, involving dysfunction and oversecretion of multiple proinflammatory molecules, chiefly tumor necrosis factor- (TNF-) α, interleukin- (IL-) 1β, and IL-6. However, although biological treatment with anti-TNF-α agents has been largely demonstrated to be effective in BD, not all patients are definite responders, and this beneficial response might drop off over time. Therefore, additional therapies for a subset of refractory patients with BD are inevitably needed. Different agents targeting various cytokines and their receptors or cell surface molecules have been studied: the IL-1 receptor has been targeted by anakinra, the IL-1 by canakinumab and gevokizumab, the IL-6 receptor by tocilizumab, the IL12/23 receptor by ustekinumab, and the B-lymphocyte antigen CD-20 by rituximab. The aim of this review is to summarize all current experiences and the most recent evidence regarding these novel approaches with biological drugs other than TNF-α blockers in BD, providing a valuable addition to the actually available therapeutic armamentarium.