Correlation of MPO-ANCA Epitope between Patients with Kawasaki Disease and Their Mothers

Abstract

Recently, the activated neutrophils and the antibodies against neutrophils, anti-neutrophil cytoplasmic antibody (ANCA), have been demonstrated in relation to the development of vasculitis. The elevation of neutrophil counts in peripheral blood in Kawasaki disease is believed to cause the development of aneurysm in coronary artery. Elevation in the levels of myeloperoxidase specific ANCA (MPO-ANCA) in sera of patients with Kawasaki disease has also been observed. In order to investigate the role of MPO-ANCA in the progression of vasculitis, epitope analysis of MPO-ANCA have been reported (1, 2). MPO-ANCA reacted with N-terminus and C-terminus in the heavy chain of MPO, suggesting correlation of specific monoclonal/oligoclonal MPO-ANCA with the progression of vasculitis. In coronary arteritis in Kawasaki disease the epitope of MPO-ANCA was also analyzed. Furthermore, we analyzed correlation of MPO-ANCA epitope(s) in sera of between patients and their mother in order to know source of the antibody. Most of healthy mothers showed MPO-ANCA positive in their sera and about epitope in sera of patients were coincident by 50% with that of mothers, but not father's. These results suggest that source of auto-antibody MPO-ANCA may be same to that of patient's mother's. 1)Tomizawa et al. J Clin. Immunol. 18, 142-152, 1998. 2) Fujii et al. Clin. Nephrology 53, 242-252, 2000.