Treating the Different Phenotypes of Behçet's Syndrome

Frontiers in Immunology

Giacomo Emmi¹  Domenico Prisco¹  Alessandro Barilaro²  Alessandra Bettiol³  Gulen Hatemi⁴  Lorenzo Vannozzi⁵ 

[1] Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy;Department of Neurology 2 and Multiple Sclerosis Regional Referral Centre, Careggi University Hospital, Florence, Italy;Department of Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy;Division of Rheumatology, Department of Internal Medicine, Istanbul University–Cerrahpaşa, Istanbul, Turkey;Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy;

关键词: Behçet's syndrome;    phenotypes;    cluster analysis;    anti-TNF-α;    DMARDs;   

DOI  :  10.3389/fimmu.2019.02830

来源: DOAJ

【 摘 要 】

Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and gastrointestinal manifestations. Based on this heterogeneity, BS can be hardly considered as a single clinical entity. Growing evidence supports that, within BS, different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, three major BS phenotypes have been reported: (a) the mucocutaneous and articular phenotype, (b) the extra-parenchymal neurological and peripheral vascular phenotype, and (c) the parenchymal neurological and ocular phenotype. To date, guidelines for the management of BS have been focused on the pharmacological treatment of each specific BS manifestation. However, tailoring the treatments on patient's specific phenotype, rather than on single disease manifestation, could represent a valid strategy for a personalized therapeutic approach to BS. In the present literature review, we summarize current evidence on the pharmacological treatments for the first-, second-, and third-line treatment of the major BS phenotypes.

【 授权许可】

Unknown