原发性小血管炎是一类自身免疫性疾病,可累及全身多个脏器。肾脏受累则多进展迅速,造成不可逆的终末期肾衰竭,肺受累则可引起大咯血而危及生命。抗中性粒细胞胞质抗体(ANCA)是其特异性的血清学诊断工具,也称之为ANCA相关性小血管炎。但及时诊断和治疗,多可控制病情进展,甚至可使病情逆转。因此,及时诊断是改善患儿预后的关键。 Vasculitis is a type of autoimmune disease that can affect multiple organs throughout the body. Kidney involvement is much more rapid progress, resulting in irreversible end-stage renal failure, pulmonary involvement can cause massive hemoptysis and life-threatening. Anti-neutrophil cytoplasmic antibodies (ANCA) is a specific serological diagnostic tool, also known as ANCA-associated vasculitis. But timely diagnosis and treatment, more control of disease progression, and even make the disease reversed. Therefore, timely diagnosis is the key to improve the prognosis of children.