Usually associated with autoimmune diseases, anti-neutrophil cytoplasmic antibodies are also detected in other conditions, such as infections, malignancies, and after intake of certain drugs. Even if the mechanisms of production and their pathogenic role have not been fully elucidated yet, ANCA are widely recognized as a clinically alarming finding due to their association with various disorders. While ANCA target several autoantigens, proteinase-3, and myeloperoxidase are the ones proved to be most frequently related to chronic inflammation and tissue damage in murine models. Albeit these autoantibodies could be present as an isolated observation without any implications, ANCA are frequently used in clinical practice to guide the diagnosis in a suspect of small vessel vasculitis. Conditions that should prompt the clinician to test ANCA status range from various forms of lung disease to renal or peripheral nervous system impairment. ANCA positivity in the presence of an autoimmune disease, especially rheumatoid arthritis, or connective tissue diseases, is frequently correlated with more clinical complications and treatment inefficacy, even in the absence of signs of vasculitis. For this reason, it has been postulated that ANCA could represent the final expression of an immune dysregulation rather than a pathogenic event responsible for organs damage. Recently, it has also been proposed that ANCA specificity (PR3 or MPO) could possibly define ANCA-associated vasculitides better than clinical phenotype. This review aims at summarizing the latest advancements in the field of ANCA study and clinical interpretation.

通常与自身免疫性疾病相关，抗中性粒细胞胞质抗体也可在其他情况下检测到，例如感染、恶性肿瘤和服用某些药物后。即使生产机制及其致病作用尚未完全阐明，但由于 ANCA 与多种疾病相关，因此被广泛认为是临床上令人担忧的发现。虽然 ANCA 靶向几种自身抗原，但在小鼠模型中，蛋白酶 3 和髓过氧化物酶被证明最常与慢性炎症和组织损伤相关。尽管这些自身抗体可能作为孤立的观察结果出现而没有任何影响，但 ANCA 在临床实践中经常用于指导疑似小血管炎的诊断。应促使临床医生检测 ANCA 状态的病症范围从各种形式的肺病到肾脏或周围神经系统损伤。存在自身免疫性疾病（尤其是类风湿性关节炎或结缔组织疾病）时的 ANCA 阳性通常与更多的临床并发症和治疗无效相关，即使没有血管炎迹象也是如此。出于这个原因，已经假设 ANCA 可能代表免疫失调的最终表达，而不是导致器官损伤的致病事件。最近，还提出 ANCA 特异性（PR3 或 MPO）可能比临床表型更好地定义 ANCA 相关血管炎。本综述旨在总结 ANCA 研究和临床解释领域的最新进展。