Abstract

The important role of B cells in the pathogenesis of systemic vasculitis has become increasingly clear over recent years. B cell directed therapies offer an exciting new approach to the treatment of these conditions, in which there has been little change in the options available to managing clinicians since the advent of traditional immunosuppressive therapy. The introduction of conventional immunosuppression has transformed survival in systemic vasculitis, particularly ANCA-associated vasculitis, but at the expense of significant toxicity, and with suboptimal efficacy. B cell therapies offer the possibility of targeted, individually tailored immunotherapy, which by improving efficacy, and reducing toxicity will improve clinical outcomes in systemic vasculitis. However, the heterogeneity of vasculitic syndromes suggests that one agent is unlikely to be effective in all situations. The precise indications and optimal combination of B cell directed with other therapies will need to be determined.

Keywords: Vasculitis, B cell, rituximab, ANCA, Aortitis, aorta, Anti-neutrophil cytoplasm antibodies, Giant cell arteritis, Polyarteritis nodosa, Churg-Strauss Syndrome