Fijolek Justyna, Sniady Anna
Third Department of Pneumonology and Oncology, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warsaw, Poland.
Medical Academy of Applied Holistic Sciences, 01-234 Warsaw, Poland.
J Clin Med. 2025 Jun 30;14(13):4631. doi: 10.3390/jcm14134631.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and interstitial lung disease (ILD) represent a complex interplay between autoimmune and fibrotic processes that poses significant diagnostic and therapeutic challenges. The distinction between isolated ANCA-ILD and AAV-ILD remains a subject of ongoing debate, with some researchers proposing that ANCA-ILD may be an early or restricted form of systemic vasculitis. Immunosuppressive therapy is the cornerstone of treatment for both diseases. However, there is increasing evidence that supports the potential role of antifibrotic agents in the management of progressive fibrosis. Management of these diseases requires a personalized approach that incorporates evaluation of biomarkers, imaging findings, and clinical risk factors to guide treatment decisions. Although current therapeutic strategies primarily target systemic inflammation, addressing the fibrotic components of these diseases is crucial for improving outcomes. Furthermore, emerging therapeutic options, such as B-cell depletion and antifibrotic therapies, offer promising outcomes. However, their roles in the treatment of AAV-ILD require further exploration. In this review, we discuss clinical insights and evolving therapeutic strategies for managing AAV and ANCA-positive ILD. In addition, we highlight the importance of early diagnosis and individualized treatment plans in improving the prognosis and quality of life of affected patients.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）和间质性肺疾病（ILD）代表了自身免疫和纤维化过程之间的复杂相互作用，这带来了重大的诊断和治疗挑战。孤立性ANCA-ILD和AAV-ILD之间的区别仍然是一个持续争论的话题，一些研究人员提出ANCA-ILD可能是系统性血管炎的早期或局限性形式。免疫抑制治疗是这两种疾病治疗的基石。然而，越来越多的证据支持抗纤维化药物在进行性纤维化管理中的潜在作用。这些疾病的管理需要一种个性化的方法，包括评估生物标志物、影像学检查结果和临床风险因素，以指导治疗决策。虽然目前的治疗策略主要针对全身炎症，但解决这些疾病的纤维化成分对于改善预后至关重要。此外，新兴的治疗选择，如B细胞清除和抗纤维化治疗，提供了有希望的结果。然而，它们在AAV-ILD治疗中的作用需要进一步探索。在这篇综述中，我们讨论了管理AAV和ANCA阳性ILD的临床见解和不断发展的治疗策略。此外，我们强调早期诊断和个性化治疗计划在改善受影响患者的预后和生活质量方面的重要性。
