Biotherapies in large vessel vasculitis

Autoimmun Rev. 2016 Jun;15(6):544-51. doi: 10.1016/j.autrev.2016.02.012. Epub 2016 Feb 12.


Giant cell arteritis (GCA) and Takayasu's arteritis (TA) are large vessel vasculitis (LVV) and aortic involvement is not uncommon in Behcet's disease (BD) and relapsing polychondritis (RP). Glucocorticosteroids are the mainstay of therapy in LVV. 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 LVV. Anti-tumor necrosis factor-α 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 LVV have been encouraging. The development of new biologic targeted therapies will probably open a promising future for patients with LVV.

Keywords: Behçet's disease; Biotherapy; Giant cell arteritis; Relapsing polychondritis; Takayasu.

Publication types

  • Review

MeSH terms

  • Behcet Syndrome / drug therapy*
  • Biological Therapy / methods*
  • Giant Cell Arteritis / drug therapy*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Polychondritis, Relapsing / drug therapy*
  • Takayasu Arteritis / drug therapy*


  • Immunosuppressive Agents