Codes-Méndez Helena, Castillo Diego, Moya-Alvarado Patricia, Giménez-Palleiro Ana, Castellvi Ivan
Department of Rheumatology.
Department of Pulmonology.
Curr Opin Pulm Med. 2025 Jun 24. doi: 10.1097/MCP.0000000000001187.
The intersection of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and interstitial lung disease (ILD) represents a complex and increasingly recognized clinical challenge. This review aims to summarize current understanding, highlight diagnostic and therapeutic approaches, and identify key gaps in the literature regarding ANCA-associated ILD.
ANCA positivity-particularly MPO-ANCA- is increasingly identified in patients with fibrotic ILD, even in the absence of systemic vasculitis. This overlap raises questions about disease classification and management, especially as radiologic patterns such as usual interstitial pneumonia (UIP) appear to predict prognosis. Immunosuppressive therapy remains the mainstay of treatment, though its role varies depending on the presence of systemic features and lung fibrosis. Emerging biomarkers, and the potential role of antifibrotic agents offer promising avenues for improved monitoring and therapy.
ANCA-ILD represents a heterogeneous and underexplored disease spectrum that challenges existing classification systems. A multidisciplinary approach is critical, and prospective studies are urgently needed to redefine diagnostic criteria and guide treatment strategies in order to improve clinical outcomes.
抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）与间质性肺疾病（ILD）的交叉领域是一个复杂且日益受到认可的临床挑战。本综述旨在总结当前的认识，突出诊断和治疗方法，并确定关于ANCA相关ILD的文献中的关键空白。
在纤维化ILD患者中，即使没有系统性血管炎，ANCA阳性，尤其是髓过氧化物酶-ANCA（MPO-ANCA）也越来越多地被发现。这种重叠引发了关于疾病分类和管理的问题，特别是因为诸如普通间质性肺炎（UIP）等放射学模式似乎可以预测预后。免疫抑制治疗仍然是主要的治疗方法，但其作用因是否存在全身特征和肺纤维化而异。新兴的生物标志物以及抗纤维化药物的潜在作用为改善监测和治疗提供了有希望的途径。
ANCA相关ILD代表了一个异质性且未被充分探索的疾病谱，对现有的分类系统提出了挑战。多学科方法至关重要，迫切需要进行前瞻性研究以重新定义诊断标准并指导治疗策略，从而改善临床结局。
