Development of Anti-neutrophil Cytoplasmic Antibodies (ANCA) and Vasculitis in BALB/c Mice: A Prototype of Human ANCA Associated Disease

To investigate whether immunization in BALB/c mice with ANCA will induce ANCA associated vasculitis that will serve as an animal model for the diseased mice were immunized with myeloperoxidase (MPO), proteinase3 (PR3) and 'a granule' extract of polymorphonuclear leukocytes (PMN) using purified human whole antibody to MPO and PR3 and also the F(ab')(2) fractions of MPO-ANCA and PR3-ANCA. Control mice were immunized with normal human IgG. Mice immunized with purified MPO and PR3 as well as with human IgG-ANCA and its F(ab')(2) fractions, developed ANCA and AAV, while control mice did not develop these autoantibodies or the disease. This ANCA could be induced in BALB/c mice by antigen and antibody immunizations and these mice showed the possible development of ANCA related vasculitis indicating a likely pathogenic role of ANCA.