Immune regulatory mechanisms in ANCA-associated vasculitides

A group of primary vasculitides is associated with the presence of anti-neutrophil cytoplasmic autoantibodies (ANCA). In these diseases, the contribution of ANCA to disease pathogenesis has been studied extensively. Also, in patients with ANCA-associated vasculitides, the T lymphocyte compartment is dysregulated, as aberrant distribution and function of T cell subsets has been shown. In this review we will discuss the putative role of T lymphocytes in inflammation and granuloma formation, as well as the involvement of B cell compartments in the pathophysiology of ANCA-associated vasculitis.