Wegener型肉芽肿病以上下呼吸道坏死性肉芽肿病变,累及小血管的播散性坏死性血管炎和灶性或增生性肾小球肾炎为主要临床特征,在耳鼻咽喉头颈外科表现复杂多变。鼻及鼻窦是头颈部中受累发生率最高的部位,外耳、中耳及内耳均可受累,声门下狭窄是其在喉及气管最主要的表现。该病诊断困难,需结合临床表现、抗中性粒细胞胞浆抗体(antineutrophilcytoplasmicantibody,ANCA)检测及病理活检等多个方面。 Wegener-type granulomatosis with upper and lower respiratory necrosis granulomatous lesions involving disseminated vascular necrosis of small vessels and focal or proliferative glomerulonephritis as the main clinical features, performance in the otolaryngology head and neck surgery complex and volatile. Nasal and sinus are the highest incidence of head and neck parts affected, the outer ear, middle ear and inner ear can be involved, subglottic stenosis in the throat and trachea is the most important performance. The diagnosis of the disease is difficult, need to be combined with clinical manifestations, anti-neutrophil cytoplasmic antibody (antineutrophilcytoplasmicantibody, ANCA) and biopsy and other aspects.