原发性抗中性粒细胞胞浆抗体(an ti neu troph il cytop las-m ic an tibod ies,ANCA)相关性小血管炎系累及全身多系统的自身免疫性疾病,此类疾病的临床表现复杂多样,故极易误诊而导致病情恶化。2006年我科收治以耳痛为首发症状的原发性ANCA相关性小血管炎1例,结合文献复习讨论 Primary anti-neutrophil cytoplasmic antibodies (ANCA) -related vasculitis is a systemic, multisystem autoimmune disease that is clinically manifest in these diseases Complex and diverse, so easily misdiagnosed and lead to deterioration of the condition. One case of primary ANCA-associated vasculitis treated with earache as the first symptom in our department in 2006, combined with literature review and discussion