Role of Anti-neutrophil Cytoplasmic Antibodies in Kawasaki Disease

Background: Kawasaki disease (KD) is characterized by systemic vasculitis and may lead to coronary artery ectasia and aneurysm. Autoantibodies, in particular anti-neutrophil cytoplasmic antibodies (ANCAs), have been identified in association with the vasculitis. The relationship between ANCAs and KD is unclear. The purpose of this study is to investigate the role of ANCAs in KD in Taiwan. Methods: We assessed ANCAs in 10 KD patients (6 male and 4 female; age range, 4 months to 3 years) between January and December 2003. Results: Cytoplasmic (c)-ANCA staining was absent in all patients, but 5 patients showed perinuclear (p)-ANCA staining. C-reactive protein levels were significantly higher in p- ANCA-positive than p-ANCA-negative KD patients (12.672±3.05 vs. 4.186±2.60 mg．dL^-1, respectively; P ＜ 0.01); there was no significant difference in age, white blood cell count, or clinical presentation. Conclusions: Our data showed that CRP levels were significantly higher in p-ANCA-positive patients and c-ANCA was negative in all KD patients. Neither c-ANCA nor p-ANCA was linked to coronary artery abnormalities in KD patients, suggesting that ANCAs may play only a minor role in the pathogenesis of KD.