PReS-FINAL-2189: Tocillizumab for patients with takayasu arteritis in childhood refractory to conventional therapy

Takayasu arteritis (TA) is a chronic systemic granulomatous vasculitis of large vessels. Corticosteroids may induce remission. However, over half of patients flare with tapering of corticosteroids. There are anecdotal reports of treatment with methotrexate, azathioprine, and biologics such as infliximab. Cyclophosphamide is recommended for life-threatening or organ-threatening patients. However, patients, especially with HLA B52-positive, are frequently refractory to these treatments. Since serum levels of interleukin (IL)-6 are correlated with disease activity, an open-label trial of tocilizumab (TCZ), an anti-IL-6 receptor monoclonal antibody, was conducted for 6 pediatric patients with TA.