Abstract: Objective: To analyse the characteristics of the anti-centromere antibody (ACA) positive patients with autoimmunity disease. Methods: The data of the autoimmunity disease patients with anti-centromere antibody (ACA) detected by indirect immunofluorescence in our hospital from January, 2020 to June 2021 were analyzed retrospectively. The patients were divided into ACA positive group and ACA negative group, and the gender, age, clinical diagnosis, antinuclear antibody spectrum, immunological index, manifestations were compared between the two group. Results: 53 cases with ACA positive and 31 cases with ACA negative were analyzed. The average age of ACA positive group was 59.85±11.79, which was higher than ACA negative group (P＜0.05). The proportion of high titer（≥1∶1000）of ACA antibody titer, anti CENP-B antibody and anti AMA-M2 antibody in ACA positive gruop was 98.11%, 94.34% and 22.64%, which were higher than those in ACA negative gruop (77.42%, 6.45% and 6.45%) (Ps＜0.05). The average serum IgG level was 11.22±5.36 g/L in ACA positive group which was lower than ACA negative group (P<0.05). The ratio of the patients with Raynaud's phenomenon, cardiovascular involvement was 43.40% and 33.96% in ACA positive group, which was higher than those in ACA negative group (P<0.05). Conclusion:The characteristics of ACA positive patients with autoimmunity disease include older onset age, higher ACA antibody titer, AMA-M2 antibody level and CENP-B antibody level, easy to complicated with Raynaud's phenomenon and cardiovascular diseases, lower serum IgG level. The pathogenesis mechanism of ACA in autoimmunity diseases deserves more deeply study.

Key words: autoimmunity diease, anti centromere antibody, AMA-M2, CENP-B