Antineutrophil Cytoplasmic Antibody-Associated Vasculitis-Related Lung Injury

ANCA相关性血管炎(ANCA-associated vasculitis, AAV)是一种以小血管受累为主，同时也伴有中血管受累，以坏死性小血管炎为主要病理表现并伴随着全身多系统累及的异质性自身免疫性疾病。作为全身性疾病，肺的累及是AAV最常见的受累器官之一，同时，肺受累也是AAV预后不佳的重要指标。本综述将对ANCA相关性血管炎肺损伤的流行病学、机制、临床特点、治疗进行讨论。ANCA-associated vasculitis (AAV) is a heterogeneous autoimmune disease with mostly small vessels involvement, but also medium vessels, and the main pathological manifestation is necrotizing small vasculitis which is accompanied with systemic multi-system involvements. As a systemic disease, the lung is one of the target organs commonly involved in AAV, which predicts increased mortality. This review discusses the epidemiology, mechanism, clinical features, and treatment of lung injury in ANCA-associated vasculitis.