Giant cell arteritis (GCA) and Takayasu's arteritis (TA) are large vessel vasculitis (LW) and aortic involvement is not uncommon in Behcet's disease (BD) and relapsing polychondritis (RP). Glucocorticosteroids are the mainstay of therapy in LW. However, a significant proportion of patients have glucocorticoid dependance, serious side effects or refractory disease to steroids and other immunosuppressive treatments such as cyclophosphamide, azathioprine, mycophenolate mofetil and methotrexate. Recent advances in the understanding of the pathogenesis have resulted in the use of biological agents in patients with LW. Anti-tumor necrosis factor-alpha drugs seem effective in patients with refractory Takayasu arteritis and vascular BD but have failed to do so in giant cell arteritis. Preliminary reports on the use of the anti-IL6-receptor antibody (tocilizumab), in LW have been encouraging. The development of new biologic targeted therapies will probably open a promising future for patients with LW. (C) 2016 Elsevier B.V. All rights reserved.
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Immanuel Krankenhaus Berlin, Med Ctr Rheumatol Berlin Buch, Lindenberger Weg 19, D-13125 Berlin, GermanyImmanuel Krankenhaus Berlin, Med Ctr Rheumatol Berlin Buch, Lindenberger Weg 19, D-13125 Berlin, Germany
Schmidt, Wolfgang A.
Nielsen, Berit D.
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Silkeborg Reg Hosp, Diagnost Ctr, Falkevej 1A, DK-8600 Silkeborg, DenmarkImmanuel Krankenhaus Berlin, Med Ctr Rheumatol Berlin Buch, Lindenberger Weg 19, D-13125 Berlin, Germany
Nielsen, Berit D.
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Univ Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, SpainUniv Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, Spain
Espigol-Frigole, Georgina
Prieto-Gonzalez, Sergio
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Univ Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, SpainUniv Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, Spain
Prieto-Gonzalez, Sergio
Alba, Marco A.
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Univ Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, SpainUniv Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, Spain
Alba, Marco A.
Tavera-Bahillo, Itziar
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Univ Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, SpainUniv Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, Spain
Tavera-Bahillo, Itziar
Garcia-Martinez, Ana
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Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Emergency Dept, Vasculitis Res Unit, Barcelona 08036, SpainUniv Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, Spain
Garcia-Martinez, Ana
Gilabert, Rosa
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Univ Barcelona, Inst Invest Biomed Pi i Sunyer IDIBAPS, Hosp Clin Barcelona, Ctr Diagnost Imaging, E-08036 Barcelona, SpainUniv Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, Spain
Gilabert, Rosa
Hernandez-Rodriguez, Jose
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Univ Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, SpainUniv Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, Spain
Hernandez-Rodriguez, Jose
Cid, Maria C.
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Univ Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, SpainUniv Barcelona, Inst Invest Biomed August Pi I Sunyer IDIBAPS, Hosp Clin Barcelona, Vasculitis Res Unit,Dept Syst Autoimmune Dis, E-08036 Barcelona, Spain