Alba Marco A, Flores-Suárez Luis Felipe, Henderson Ashley G, Xiao Hong, Hu Peiqi, Nachman Patrick H, Falk Ronald J, Charles Jennette J
Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Primary Systemic Vasculitis Clinic, Instituto Nacional de Enfermedades Respiratorias (INER), Mexico City, Mexico.
Autoimmun Rev. 2017 Jul;16(7):722-729. doi: 10.1016/j.autrev.2017.05.008. Epub 2017 May 4.
Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides are immune-mediated disorders that primarily affect small blood vessels of the airway and kidneys. Lung involvement, one of the hallmarks of microscopic polyangiitis and granulomatosis with polyangiitis, is associated with increased mortality and morbidity. In recent years, several retrospective series and case reports have described the association of interstitial lung disease (ILD) and ANCA vasculitis, particularly those positive for ANCA specific for myeloperoxidase. In the majority of these patients pulmonary fibrosis occurs concurrently or predates the diagnosis of ANCA vasculitis. More importantly, these studies have shown that ILD has an adverse impact on the long-term prognosis of ANCA vasculitis. This review focuses on the main clinical and radiologic features of pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibodies. Major histopathology features, prognosis and therapeutic options are summarized.
抗中性粒细胞胞浆抗体（ANCA）血管炎是一种免疫介导的疾病，主要影响气道和肾脏的小血管。肺部受累是显微镜下多血管炎和肉芽肿性多血管炎的特征之一，与死亡率和发病率增加相关。近年来，一些回顾性系列研究和病例报告描述了间质性肺疾病（ILD）与ANCA血管炎的关联，特别是那些髓过氧化物酶特异性ANCA阳性的患者。在这些患者中，大多数肺纤维化与ANCA血管炎同时发生或在其诊断之前出现。更重要的是，这些研究表明ILD对ANCA血管炎的长期预后有不利影响。本综述重点关注抗中性粒细胞胞浆抗体相关肺纤维化的主要临床和放射学特征。总结了主要组织病理学特征、预后和治疗选择。
