A new experimental model for Wegener's granulomatosis

Wegener's granulomatosis (WG) is a granulomatous vasculitis that affects primarily the upper respiratory track, lungs and kidneys. WG is considered an autoimmune disease since it is closely associated with specific autoantibody, namely antineutrophil cytoplasmic antibodies (ANCA), directed against proteinase 3, a 29 kDa serine protease of neutrophil primary granules. However, despite the existence of abundant indirect evidence for the involvement of ANCA in the pathogenesis of WG, direct evidence for the pathogenicity of ANCA is lacking. This paper uses mice as a model for the induction of autoimmunity by idiotic manipulation to try and provide evidence for the pathoginicity of ANCA. Results indicate that the ability to induce WG in mice by immunizing them with ANCA provides direct evidence for their pathogenicity and can be used as a tool to further study the mechanisms of the disease.