Blackabey Victoria, Gan Richard Wei Chern, Buglass Helen, Kaul Veena, Ward Victoria M M
Department of Otolaryngology, Pinderfields General Hospital, Wakefield, UK.
AME Case Rep. 2018 Apr 30;2:17. doi: 10.21037/acr.2018.03.01. eCollection 2018.
Granulomatosis with polyangiitis (GPA) is characterised by vasculitis of small and medium sized blood vessels and granulomatous lesions of the respiratory tract. The aetiology is unclear, however it is thought to be due to an autoimmune process with about 92% of patients with the disease being antineutrophilic cytoplasmic antibodies (ANCA) positive. Patients normally present in early adulthood, more commonly in the winter months. Seventy percent of patients with GPA present with ear, nose or throat symptoms. These include nasal congestion, crusting, epistaxis, nasal septal perforation and nasal saddle deformity. Lesions in the airway can lead to subglottic stenosis with resultant airway obstruction. Treatment of the disease complicated by subglottic stenosis is not straightforward and the benefits and risks of options including medical and surgical management need to be weighed up and tailored to each individual case. We describe two cases of GPA complicated by airway obstruction due to subglottic stenosis and their management.
肉芽肿性多血管炎（GPA）的特征是中小血管血管炎和呼吸道肉芽肿性病变。病因尚不清楚，但认为是由自身免疫过程引起的，约92%的该疾病患者抗中性粒细胞胞浆抗体（ANCA）呈阳性。患者通常在成年早期发病，更常见于冬季。70%的GPA患者有耳、鼻或喉症状。这些症状包括鼻塞、结痂、鼻出血、鼻中隔穿孔和鼻背畸形。气道病变可导致声门下狭窄，进而导致气道阻塞。因声门下狭窄并发该疾病的治疗并不简单，包括药物和手术治疗在内的各种选择的益处和风险需要权衡，并根据每个病例进行调整。我们描述了两例因声门下狭窄导致气道阻塞的GPA病例及其治疗情况。
