探讨以鼻部症状为首发症状的抗中性粒细胞胞浆抗体相关性血管炎（AAV）的临床特征、治疗方案和预后。

回顾分析2019年1月至2024年12月就诊于徐州医科大学附属淮安医院耳鼻喉头颈外科的3例AAV患者的临床资料、诊治经过及随访结果。

3例AAV患者均以慢性鼻窦炎症状首发，反复鼻出血1例，鼻塞伴头痛2例，3例患者均有肺结节、局部钙化或间质性改变等肺部表现；其中第1例反复鼻出血入院，后鼻窦CT确诊为慢性鼻窦炎予以鼻内镜手术，术后仍有鼻出血、头部及眼眶等疼痛不能缓解，经进一步抗中性粒细胞胞浆抗体（ANCA）相关免疫学指标检查确诊，转风湿免疫科正规治疗后好转，近期鼻部症状又有反复；第2例以鼻窦炎首诊入院，鼻内镜微创手术后鼻塞、头疼明显好转，后因反复咳嗽不能缓解，遂检查后确诊ANCA，予以正规治疗后好转；第3例以慢性鼻窦炎、鼻腔肿物首诊于外院行鼻内镜手术，术后鼻塞、头疼等持续存在，抗炎等治疗无效后，经风湿免疫科会诊确诊，予以环磷酰胺、激素等治疗后好转出院。3例患者目前均仍在随访中。

以鼻窦炎表现为首发症状的AAV临床主要表现为鼻塞、头疼、鼻出血、鼻溃疡、鼻息肉等，耳鼻咽喉头颈外科医生容易先入为主予以鼻内镜手术等治疗，从而导致误诊误治；抗中性粒细胞胞浆抗体谱检测有助于病情诊断,同时还需结合临床表现、病理、免疫学指标等综合分析，以期早期确诊。

To investigate the clinical features, treatment strategies, and prognosis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with nasal symptoms as the initial manifestation.

A retrospective analysis was conducted on three AAV patients treated at the Department of Otolaryngology-Head and Neck Surgery, Huai’an Hospital Affiliated to Xuzhou Medical University between January 2019 and December 2024. Clinical data, diagnostic and therapeutic interventions, and follow-up outcomes were systematically reviewed.

All patients initially exhibited chronic sinusitis-like symptoms: recurrent epistaxis (n=1) and nasal obstruction with headache (n=2). Pulmonary manifestations (lung nodules, localized calcification, or interstitial changes) were observed in all cases. Case 1 was hospitalized for recurrent epistaxis and diagnosed with chronic sinusitis via sinus CT, and he underwent endoscopic sinus surgery (ESS). Postoperative persistence of epistaxis, headache, and orbital pain prompted ANCA serology testing, which confirmed AAV. Immunosuppressive therapy initiated after rheumatology referral achieved symptom remission, though nasal symptoms recurred during follow-up. Case 2 presented with sinusitis. His nasal obstruction and headache improved after minimally invasive ESS. Persistent cough led to ANCA detection and subsequent immunosuppressive treatment with resolution of symptoms. Case 3 was initially diagnosed with chronic sinusitis and a nasal mass at an external institution. ESS failed to alleviate nasal obstruction/headache. Anti-inflammatory therapy was ineffective. Rheumatology consultation confirmed AAV. Cyclophosphamide and corticosteroid treatment resulted in clinical improvement.

AAV with initial sinonasal involvement typically manifests as nasal obstruction, headache, epistaxis, nasal ulceration, and/or polyposis. Otolaryngologists may reflexively resort to ESS for symptom management, potentially resulting in delayed diagnosis and inappropriate initial management. While ANCA serology testing provides critical diagnostic support, definitive diagnosis requires integrated assessment of clinical manifestations, histopathological findings (where available), and immunological parameters to facilitate early disease confirmation. Multidisciplinary collaboration is essential to mitigate diagnostic errors.