Department of ENT, Salford Care Organisation, Northern Care Alliance NHS foundation Trust, Salford, UK
Department of ENT, Tameside and Glossop IC NHS Foundation trust, Ashton-under-Lyne, UK.
BMJ Case Rep. 2024 Sep 28;17(9):e258391. doi: 10.1136/bcr-2023-258391.
Granulomatosis polyangiitis (GPA) is an autoimmune condition causing inflammation of small blood vessels. It is a rare disorder that may affect various parts of the body. The diagnosis is often based on clinical examination, laboratory investigations and tissue biopsy. In about 10-20% of patients, the anti-neutrophilic cytoplasmic antibody (ANCA) can be negative, and histology maybe inconclusive, which can lead to diagnostic uncertainty. Failure to treat vasculitis can lead to morbidity and even mortality. We present a case report of a gentleman who was presented with an airway emergency with inflammation of the nasal cavity and subglottic involvement amounting to airway stenosis. His ANCA was negative and tissue biopsy from the subglottis was inconclusive. He underwent urgent dilatation of his airway, local therapies to the nose and was commenced on 10 cycles of cyclophosphamide. A follow-up of over 4 years has not shown any relapse of his disease clinically or biochemically. We discuss the clinical findings, diagnostic dilemma and multidisciplinary management of this life-threatening condition.
肉芽肿性多血管炎（GPA）是一种自身免疫性疾病，可导致小血管炎症。它是一种罕见的疾病，可能影响身体的各个部位。诊断通常基于临床检查、实验室检查和组织活检。约 10-20%的患者抗中性粒细胞胞质抗体（ANCA）可能为阴性，组织学可能不确定，这可能导致诊断不确定。未能治疗血管炎会导致发病率甚至死亡率。我们报告了一例男性患者，他因气道紧急情况就诊，鼻腔炎症和会厌下受累导致气道狭窄。他的 ANCA 为阴性，会厌下的组织活检结果不确定。他接受了紧急气道扩张、鼻部局部治疗，并开始接受 10 个周期的环磷酰胺治疗。超过 4 年的随访未显示出任何疾病的临床或生化复发。我们讨论了这种危及生命的疾病的临床发现、诊断困境和多学科管理。
