Martinez Del Pero M, Sivasothy P
Department of ENT, Addenbrooke's Hospital, Cambridge, UK.
Best Pract Res Clin Rheumatol. 2009 Jun;23(3):403-17. doi: 10.1016/j.berh.2009.02.003.
The pulmonary vasculitides are a heterogeneous group of rare disorders that result from an inflammatory process damaging the vessel wall and consequent impaired blood flow, ischaemia and tissue necrosis. The clinical manifestation of these vasculitides depends on the site, size, type and severity of the inflammatory process. Vasculitis involving the airways is a common feature of the anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitides (AASVs) and can predate the diagnosis by years. Secondary causes of vasculitis associated with connective tissue disorders are also capable of presenting with pulmonary features. Recognition of involvement, investigation and treatment are important to ameliorate symptoms for patients. This article concentrates on the assessment and specific management of upper and lower airway problems of AASV.
肺血管炎是一组异质性罕见疾病，由炎症过程损伤血管壁并导致血流受损、缺血和组织坏死引起。这些血管炎的临床表现取决于炎症过程的部位、大小、类型和严重程度。累及气道的血管炎是抗中性粒细胞胞浆抗体（ANCA）相关系统性血管炎（AASV）的常见特征，可在诊断前数年出现。与结缔组织疾病相关的血管炎的继发性病因也可表现出肺部特征。认识到受累情况、进行检查和治疗对改善患者症状很重要。本文重点关注AASV上下气道问题的评估和具体管理。
