title: Current and future treatments for Behçet's uveitis: road to remission.
abstract: Behçet's disease (BD) is a multisystem inflammatory disorder of uncertain origin, although it remains defined within the spectrum of systemic immune-mediated vasculitic disorders and also represents a spectrum of putative autoimmune disease. Major symptoms include oral aphthous ulcers, genital ulcerations, skin lesions, and ocular lesions. Despite afflicting many systems, ocular complications of BD are some of the more devastating for the patient and their quality of life. Eye involvement, which affects 60-80 % of BD patients, is characterized in its more severe form by posterior or panuveitis including occlusive retinal vasculitis. While pathogenesis of BD remains complex, association with Class I MHC (HLA-B*51) predisposing to inflammation with engagement of the innate-immune system (neutrophils, NK cells), and perpetuated by the adaptive T cell responses against infectious- and/or auto-antigens. Despite the choice of conventional immunosuppressive therapies available, only recently with the advent of biologic therapy has visual prognosis and outcomes been substantially and favorably altered. For example, both interferon-α (IFN-α) and tumour necrosis factor (TNF)-α antagonists deliver promising results and for the first time improve prognosis. With IFN-α therapy, durable remissions of uveitis can be achieved and lead to drug-free remission. Similarly, anti-TNF therapy with infliximab is reported to be rapidly effective in inducing and maintaining remission. Most recently, rising evidence reports on the use of adalimumab, etanercept, and golimumab, while use of anti-interleukin (IL)-1 agents (anakinra, canakinumab, gevokizumab), IL-6 blockers (tocilizumab), and rituximab (depleting anti-CD20 antibody) is also increasing. The aim of this review is to provide evidence for the role of conventional therapies combined with evidence for advantages and disadvantages of biologic therapies in the treatment of ocular BD. Although randomized controlled trials remain sparse, evidence remains strong and enticing that biologic agents are invaluable for the treatment of sight-threatening Behçet's uveitis and makes it an exciting time for Behçet's specialists worldwide.
authors.affiliation: Ophthalmology Department, Hospital Clinic of Barcelona, University of Barcelona, Sabino de Arana, 1, 08028, Barcelona, Spain, mesquida@clinic.ub.es.
authors.forename: Marina
authors.initials: M
authors.lastname: Mesquida
authors.forename: Blanca
authors.initials: B
authors.lastname: Molins
authors.forename: Victor
authors.initials: V
authors.lastname: Llorenç
authors.forename: María Victoria
authors.initials: MV
authors.lastname: Hernández
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Dick
authors.forename: Alfredo
authors.initials: A
authors.lastname: Adán
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001528:Behcet Syndrome; D001685:Biological Factors / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007372:Interferons / Q000627:therapeutic use; D014605:Uveitis / Q000188:drug therapy* / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones; D001685:Biological Factors; D007166:Immunosuppressive Agents; D007372:Interferons