Interstital lung disease in ANCA vasculitis.

Interstital lung disease in ANCA vasculitis.
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DOI:
10.1016/j.autrev.2017.05.008
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发表时间:
2017-07
影响因子:
13.6
通讯作者:
Charles Jennette J
Charles Jennette J
中科院分区:
医学1区
文献类型:
--
作者:
Alba MA;Flores-Suárez LF;Henderson AG;Xiao H;Hu P;Nachman PH;Falk RJ;Charles Jennette J

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抗中性粒细胞细胞质抗体(ANCA)血管阻塞是免疫介导的疾病,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多血管炎肉芽肿病的标志之一,与死亡率和发病率增加有关。近年来,一些回顾性研究和病例报告描述了间质性肺疾病(ILD)和ANCA血管炎的关系,特别是那些ANCA特异性髓过氧化物酶阳性的病例。在这些患者中,大多数肺纤维化同时发生或早于ANCA血管炎的诊断。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。本文综述了与抗中性粒细胞细胞质抗体相关的肺纤维化的主要临床和影像学特征。总结了主要的组织病理学特征、预后和治疗选择。
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.
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