Background: No study has provided evidence of the clinical significance of the antineutrophil cytoplasmic antibody (ANCA) in patients with Takayasu arteritis (TAK). Therefore, we investigated the frequency of ANCA positivity and its clinical implications in patients with TAK. Methods: We retrospectively reviewed the medical records of 121 patients with established TAK, who had results for ANCA status at diagnosis. We collected demographic and clinical data and the ANCA results at diagnosis. Additionally, we obtained information on patients’ medications and complications during follow-up. Early coronary arterial occlusive disease (CAOD) and late CAOD were defined based on a 30-day interval after TAK classification. The chi-square test, Fisher's exact test, Mann–Whitney test, and Kaplan–Meier survival analysis were used to analyze the data. Results: The patients’ mean age was 44.6 years, and 21 patients were men (17.4%). ANCA was detected in 8 patients (6.6%), of which 2 had both the myeloperoxidase ANCA (or perinuclear ANCA) and proteinase 3 ANCA (or cytoplasmic ANCA). Early CAOD was observed in 10 patients (8.3%), and late CAOD was found in 9 patients (7.4%). In the comparative analysis, the proportion of late CAOD exhibited a tendency to increase in the ANCA-positive group compared to that in the ANCA-negative group. Kaplan–Meier analysis showed that patients with ANCA exhibited a lower cumulative late CAOD-free survival rate than those without ANCA (p = 0.012). When the algorithm for the classification of ANCA-associated vasculitis (AAV) proposed by the European Medicine Agency in 2007 was applied to 8 patients with ANCA, all were not reclassified as having AAV. Conclusions: ANCA can be detected in a minority of patients with established TAK, and it may not contribute to the reclassification of AAV. Furthermore, ANCA positivity may be associated with late CAOD in patients with TAK.
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© 2020 Japanese College of Cardiology
All Science Journal Classification (ASJC) codes
- Cardiology and Cardiovascular Medicine