关键词: 肺血管炎, 儿童, 肺泡出血, 糖皮质激素, 免疫抑制剂

Abstract: Pulmonary vasculitis is a group of diseases，which are characterized by the inflammation and destruction of pulmonary vessel walls. Usually，it is one of the pulmonary manifestations of the sysetmic disease，whereas it can also be the isolated pulmonary vascular involvement. In children，it’s rare and caused by a variety of causes. Due to the diversity of affected vessels，pulmonary vasculitis is highly heterogenous in the respiratory manifestations，and may result in pulmonary arterial stenosis，pulmonary arterial aneurysm，pulmonary arterial thrombosis，pulmonary infarction，alveolar hemorrhage，pulmonary arterial hypertension and so on. The aim of therapy is to control the vascular inflammation and manage the pulmonary complications. Usually，glucocorticoid is cornerstone in the treatment of primary pulmonary vasculitis. The combination therapy of glucocorticoid with immunosuppressant is critical to improving the prognosis. Recently，biologics have showed a good effect in the treatment of pulmonary vasculitis.

Key words: pulmonary vasculitis, child, alveolar hemorrhage, glucocorticoid, immunosuppressant