Casal Ana, Díaz-Garel Juan, Pereiro Tara, Toubes María E, Ricoy Jorge, Valdés Luis
Interdisciplinary Research Group in Pneumology, Institute of Sanitary Research of Santiago de Compostela (IDIS), Santiago de Compostela, Spain.
J Thorac Dis. 2018 Sep;10(9):5560-5575. doi: 10.21037/jtd.2018.08.117.
Systemic vasculitides frequently affect the pulmonary vasculature. As the signs and symptoms of pulmonary vasculitis are variable and nonspecific, diagnosis and treatment represent a real challenge. Vasculitides should be given consideration, as these diseases present severe manifestations of rapidly progressing pulmonary disease. Examining other organs usually affected by vasculitides (e.g., the skin and kidneys) and determining autoantibody levels are essential to a better management of the disease. A radiological study would also contribute to establishing a diagnosis. The lungs are commonly involved in small-vessel vasculitis, anti-glomerular basement membrane disease, and vasculitides associated with antineutrophil cytoplasmic antibodies. Associated life-threatening diffuse alveolar haemorrhages and irreversible damage to other organs-usually the kidneys-are severe complications that require early diagnosis. Vasculitides are rare diseases that affect multiple organs. An increasing number of treatments-including biological agent-based therapies-requiring cooperation between specialists and centers have become available in the recent years. In the same way, clinicians should be familiar with the complications associated with immunosuppressive therapies.
系统性血管炎常累及肺血管。由于肺血管炎的体征和症状多变且无特异性，诊断和治疗颇具挑战。应考虑血管炎，因为这些疾病会出现快速进展性肺部疾病的严重表现。检查通常受血管炎影响的其他器官（如皮肤和肾脏）并测定自身抗体水平对于更好地管理该疾病至关重要。影像学研究也有助于确诊。肺部通常受累于小血管炎、抗肾小球基底膜病以及与抗中性粒细胞胞浆抗体相关的血管炎。相关的危及生命的弥漫性肺泡出血和对其他器官（通常是肾脏）的不可逆损害是需要早期诊断的严重并发症。血管炎是累及多个器官的罕见疾病。近年来，越来越多的治疗方法（包括基于生物制剂的疗法）可供使用，这些治疗需要专家和中心之间的合作。同样，临床医生应熟悉与免疫抑制疗法相关的并发症。
