Pathogenesis of antineutrophil cytoplasmic autoantibody vasculitis

Pathogenesis of antineutrophil cytoplasmic autoantibody-associated vasculitis and potential targets for biologic treatment.

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are autoimmune diseases in which the small vessels are inflamed. Clinical observations suggest a pathogenic role for ANCA. Such a role is supported by in vitro experimental data and animal models, particularly for myeloperoxidase-ANCA. An in vivo pathogenic role of ANCA directed to proteinase 3 has, however, not been f...

The neutrophil in antineutrophil cytoplasmic autoantibody-associated vasculitis.

Necrotizing, small-vessel vasculitis develops in patients with circulating ANCAs. Neutrophils and monocytes harbor the two major ANCA antigens, PR3 and MPO. A whole body of in vitro experiments implicated ANCA-activated neutrophil effector functions in the pathogenesis of vasculitis, whereas the role of monocytes is less well-characterized. Mouse models for anti-MPO-induced vasculitis were deve...

The Pathogenesis of Antineutrophil Cytoplasmic Antibody Renal Vasculitis

The vasculitides comprise a heterogeneous group of diseases characterized by inflammation and destruction of blood vessels. Vessels of any size can be involved which explains the diverse spectrum of clinical diseases attributed to vasculitis. While the immunological basis of disease for vasculitis was recognized over thirty years ago,[1] a standardized classification system was only adopted nea...

Editorial: Can an Increase in Antineutrophil Cytoplasmic Autoantibody Titer Predict Relapses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis?

Treatment of antineutrophil cytoplasmic antibody-associated vasculitis.

PURPOSE OF REVIEW The primary idiopathic small-vessel vasculitis syndromes include granulomatosis with polyangiitis, Churg-Strauss syndrome, and microscopic polyangiitis. These disorders are commonly referred to as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and prominently affect the pulmonary vasculature. Although significant progress has been made in the management of ...