Department of Medicine, Yonsei University College of Medicine, Seoul, Korea (Republic of).
Division of Rheumatology, Department of Internal Medicine, Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, 03722, Seoul, Korea (Republic of).
Z Rheumatol. 2022 Oct;81(8):705-711. doi: 10.1007/s00393-021-00998-1. Epub 2021 Apr 27.
Antineutrophil cytoplasmic antibody (ANCA) is a specific autoantibody for ANCA-associated vasculitis (AAV). However, ANCA can be detected in various diseases other than AAV. Hence, in this study, we investigated and provided the name of diseases with repeated ANCA positivity and the frequency of each disease other than AAV.
We retrospectively screened the results of the tests of ANCA in 26,499 patients using the Clinical Data Repository System and included in this study only 173 patients with repeated ANCA positivity more than twice. 'Unclassifiable ANCA' was defined when ANCA was detected in patients with diseases other than AAV. 'Unclassifiable repeated ANCA' was also defined when unclassifiable ANCA was successively detected more than twice.
Among rheumatic and autoimmune diseases, the most common disease with unclassifiable repeated ANCA was vasculitis undetermined (21.0%). In terms of cardiovascular and cerebrovascular diseases, the most common disease with unclassifiable repeated ANCA was atherosclerotic heart disease (12.1%). In terms of disorders in liver, kidneys and lungs, the most common disease with unclassifiable repeated ANCA was chronic kidney disease (51 cases, 29.5%). In addition, among infections with confirmed infectious pathogens, the most common pathogen with unclassifiable repeated ANCA was varicella-zoster virus (6.9%) followed by Candida (4.6%).
Overall, regardless of category, the common diseases with unclassifiable repeated ANCA were chronic kidney disease followed by interstitial lung disease and vasculitis undetermined. Thus, we carefully suggest that physicians should pay more attention to the development of AAV or vasculitis other than AAV and, furthermore, kidneys and lungs should be monitored regularly and closely in patients with unclassifiable repeated ANCA.
抗中性粒细胞胞质抗体（ANCA）是抗中性粒细胞胞质抗体相关性血管炎（AAV）的特异性自身抗体。然而，ANCA 也可在 AAV 以外的多种疾病中被检测到。因此，在本研究中，我们调查并提供了具有重复 ANCA 阳性的疾病名称以及除 AAV 以外的每种疾病的频率。
我们使用临床数据存储系统回顾性筛选了 26499 例患者的 ANCA 检测结果，并仅纳入了重复 ANCA 阳性两次以上的 173 例患者。将在除 AAV 以外的疾病中检测到 ANCA 的情况定义为“未分类 ANCA”。当未分类 ANCA 连续两次以上被检测到时，也定义为“未分类重复 ANCA”。
在风湿和自身免疫性疾病中，最常见的具有未分类重复 ANCA 的疾病是未分类血管炎（21.0%）。在心血管和脑血管疾病中，最常见的具有未分类重复 ANCA 的疾病是动脉粥样硬化性心脏病（12.1%）。在肝脏、肾脏和肺部疾病中，最常见的具有未分类重复 ANCA 的疾病是慢性肾脏病（51 例，29.5%）。此外，在已确认感染病原体的感染中，最常见的具有未分类重复 ANCA 的病原体是水痘-带状疱疹病毒（6.9%），其次是念珠菌（4.6%）。
总体而言，无论分类如何，具有未分类重复 ANCA 的常见疾病是慢性肾脏病，其次是间质性肺病和未分类血管炎。因此，我们建议医生应更加注意 AAV 或除 AAV 以外的血管炎的发展，并且，对于具有未分类重复 ANCA 的患者，应定期密切监测肾脏和肺部。
