Treating the Different Phenotypes of Behçet's Syndrome

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 evi...

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Main Authors: Alessandra Bettiol, Gulen Hatemi, Lorenzo Vannozzi, Alessandro Barilaro, Domenico Prisco, Giacomo Emmi
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-12-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fimmu.2019.02830/full
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author Alessandra Bettiol
Alessandra Bettiol
Gulen Hatemi
Lorenzo Vannozzi
Alessandro Barilaro
Domenico Prisco
Giacomo Emmi
author_facet Alessandra Bettiol
Alessandra Bettiol
Gulen Hatemi
Lorenzo Vannozzi
Alessandro Barilaro
Domenico Prisco
Giacomo Emmi
author_sort Alessandra Bettiol
collection DOAJ
description 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.
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spelling doaj.art-bb249ba736884a75b996431bdd1e49472022-12-22T01:17:22ZengFrontiers Media S.A.Frontiers in Immunology1664-32242019-12-011010.3389/fimmu.2019.02830490693Treating the Different Phenotypes of Behçet's SyndromeAlessandra Bettiol0Alessandra Bettiol1Gulen Hatemi2Lorenzo Vannozzi3Alessandro Barilaro4Domenico Prisco5Giacomo Emmi6Department of Experimental and Clinical Medicine, University of Florence, Florence, ItalyDepartment of Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, ItalyDivision of Rheumatology, Department of Internal Medicine, Istanbul University–Cerrahpaşa, Istanbul, TurkeyEye Clinic, Careggi Teaching Hospital, University of Florence, Florence, ItalyDepartment of Neurology 2 and Multiple Sclerosis Regional Referral Centre, Careggi University Hospital, Florence, ItalyDepartment of Experimental and Clinical Medicine, University of Florence, Florence, ItalyDepartment of Experimental and Clinical Medicine, University of Florence, Florence, ItalyBehçet'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.https://www.frontiersin.org/article/10.3389/fimmu.2019.02830/fullBehçet's syndromephenotypescluster analysisanti-TNF-αDMARDs
spellingShingle Alessandra Bettiol
Alessandra Bettiol
Gulen Hatemi
Lorenzo Vannozzi
Alessandro Barilaro
Domenico Prisco
Giacomo Emmi
Treating the Different Phenotypes of Behçet's Syndrome
Frontiers in Immunology
Behçet's syndrome
phenotypes
cluster analysis
anti-TNF-α
DMARDs
title Treating the Different Phenotypes of Behçet's Syndrome
title_full Treating the Different Phenotypes of Behçet's Syndrome
title_fullStr Treating the Different Phenotypes of Behçet's Syndrome
title_full_unstemmed Treating the Different Phenotypes of Behçet's Syndrome
title_short Treating the Different Phenotypes of Behçet's Syndrome
title_sort treating the different phenotypes of behcet s syndrome
topic Behçet's syndrome
phenotypes
cluster analysis
anti-TNF-α
DMARDs
url https://www.frontiersin.org/article/10.3389/fimmu.2019.02830/full
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