The progress in the study of mechamism of anti-neutrophil cytoplasmic antibodies in pathogenesis of antineutrophil cytoplasmic vasculitis

Abstract

The antineutrophil cytoplasmic antibody associated vasculitis (AAVs) are composed of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA)and eosinophilic granulomatosis with polyangiitis (EGPA). Among them, GPA is primarily associated with anti-protease 3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA), while MPA and EGPA are mainly associated with myeloperoxidase-antineutrophil cytoplasmic antibody(MPO-ANCA) antibody.Genetic and environmental factors are involved in the pathogenesis.PR3-ANCA and MPO-ANCA are associated with different HLA class II antigens in patients, indicating that ANCAs are involved in the pathogenesis of the diseases.It is also possible that GPA and MPA are different.In vitro and in vivo data show that ANCA can induce necrotic vasculitis and glomerulonephritis, and complement-activated bypass pathway is also involved the disease.In addition, studies have found that T cells are also involved in the development ofkidneylesions.Rituximab can improve the recurrence of the kidney diseases, suggesting that B cells may also involved.Further study about the role of AAV will provide more targeted treatment for the patients with these life-threatening diseases. Key words: Anti-neutrophil cytoplasmic antibodies; Vasculitis