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Objective: The study aims to evaluate the role of anti-high mobility group box 1 (HMGB1) antibody and anti-moesin antibody in the diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and its possible relationship with the different clinical manifestations., Methods: The study involved 60 AAV patients, 58 patients with autoimmune disease other than AAV and 50 healthy subjects. The serum levels of anti-HMGB1 and anti-moesin antibodies were determined by enzyme-linked immunosorbent assay (ELISA), and the second determination was made 3 months after treatment of AAV patients., Results: Serum levels of anti-HMGB1 and anti-moesin antibodies in AAV group were significantly higher than those in non-AAV group and HC group. The area under the curve (AUC) of anti-HMGB1 and anti-moesin in diagnosing AAV were 0.977 and 0.670, respectively. Anti-HMGB1 levels were significantly elevated in AAV patients with pulmonary involvement, while the concentrations of anti-moesin were significantly increased in patients with renal damage. Anti-moesin were positively correlated with BVAS (r=0.261, P=0.044), creatinine (r=0.296, P=0.024) and negatively correlated with complement C3 (r=-0.363, P=0.013). Besides, anti-moesin levels of active AAV patients were significantly higher than those in inactive patients. The concentrations of serum anti-HMGB1 could be significantly decreased after induction remission treatment (P<0.05)., Conclusion: Anti-HMGB1 and anti-moesin antibodies play important roles in the diagnosis and prognosis of AAV, which may act as potential disease markers for AAV., (Copyright © 2023 Elsevier España, S.L.U. All rights reserved.)