National Jewish Health, Denver, CO 80206, USA.
Ther Adv Respir Dis. 2012 Dec;6(6):375-90. doi: 10.1177/1753465812454693. Epub 2012 Aug 9.
The pulmonary vasculitides are a heterogeneous group of disorders characterized pathologically by vascular destruction with cellular inflammation and necrosis. These disorders can affect small, medium, and large vessels and may be primary or occur secondary to a variety of conditions. Vasculitis involving the lungs is most commonly due to primary, idiopathic, small-vessel antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, which includes granulomatosis with polyangiitis (formerly Wegener's granulomatosis), Churg-Strauss syndrome, and microscopic polyangiitis. From a clinical perspective these remain among the most challenging of diseases both in terms of diagnosis and treatment. This review will focus on diagnosis and management of ANCA-associated vasculitides.
肺血管炎是一组异质性疾病，其病理特征为血管破坏伴细胞炎症和坏死。这些疾病可影响小、中、大血管，并且可能是原发性的，也可能继发于多种疾病。累及肺部的血管炎最常见于原发性、特发性、小血管抗中性粒细胞胞浆抗体（ANCA）相关性血管炎，包括肉芽肿性多血管炎（以前称为韦格纳肉芽肿病）、Churg-Strauss 综合征和显微镜下多血管炎。从临床角度来看，这些疾病在诊断和治疗方面都是最具挑战性的疾病之一。这篇综述将重点介绍 ANCA 相关性血管炎的诊断和治疗。
