巨细胞动脉炎相关的声门下狭窄和支气管内疾病。
Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis.
机构信息
Division of Rheumatology, MedStar Georgetown University Hospital, Washington, DC, USA.
Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD, USA.
出版信息
Rheumatology (Oxford). 2019 Dec 1;58(12):2203-2211. doi: 10.1093/rheumatology/kez217.
OBJECTIVES
To describe tracheobronchial disease in patients with granulomatosis with polyangiitis (GPA) and evaluate the utility of dynamic expiratory CT to detect large-airway disease.
METHODS
Demographic and clinical features associated with the presence of subglottic stenosis (SGS) or endobronchial involvement were assessed in a multicentre, observational cohort of patients with GPA. A subset of patients with GPA from a single-centre cohort underwent dynamic chest CT to evaluate the airways.
RESULTS
Among 962 patients with GPA, SGS and endobronchial disease were identified in 95 (10%) and 59 (6%) patients, respectively. Patients with SGS were more likely to be female (72% vs 53%, P < 0.01), younger at time of diagnosis (36 vs 49 years, P < 0.01), and have saddle-nose deformities (28% vs 10%, P < 0.01), but were less likely to have renal involvement (39% vs 62%, P < 0.01). Patients with endobronchial disease were more likely to be PR3-ANCA positive (85% vs 66%, P < 0.01), with more ENT involvement (97% vs 77%, P < 0.01) and less renal involvement (42% vs 62%, P < 0.01). Disease activity in patients with large-airway disease was commonly isolated to the subglottis/upper airway (57%) or bronchi (32%). Seven of 23 patients screened by dynamic chest CT had large-airway pathology, including four patients with chronic, unexplained cough, discovered to have tracheobronchomalacia.
CONCLUSION
SGS and endobronchial disease occur in 10% and 6% of patients with GPA, respectively, and may occur without disease activity in other organs. Dynamic expiratory chest CT is a potential non-invasive screening test for large-airway involvement in GPA.
目的
描述肉芽肿性多血管炎（GPA）患者的气管支气管疾病，并评估动态呼气 CT 检测大气道疾病的效用。
方法
在一项多中心观察性 GPA 患者队列研究中，评估与声门下狭窄（SGS）或支气管内受累相关的人口统计学和临床特征。来自单个中心队列的 GPA 患者亚组接受了动态胸部 CT 检查以评估气道。
结果
在 962 例 GPA 患者中，分别有 95 例（10%）和 59 例（6%）患者存在 SGS 和支气管内疾病。SGS 患者更可能为女性（72%比 53%，P < 0.01），诊断时年龄更小（36 岁比 49 岁，P < 0.01），且存在鞍鼻畸形（28%比 10%，P < 0.01），但更不可能有肾脏受累（39%比 62%，P < 0.01）。支气管内疾病患者更可能为 PR3-ANCA 阳性（85%比 66%，P < 0.01），有更多的 ENT 受累（97%比 77%，P < 0.01）和较少的肾脏受累（42%比 62%，P < 0.01）。大气道疾病患者的疾病活动通常局限于声门下/上气道（57%）或支气管（32%）。在通过动态胸部 CT 筛查的 23 例患者中，有 7 例有大气道病变，包括 4 例患有慢性、原因不明的咳嗽，发现有气管支气管软化症。
结论
GPA 患者中分别有 10%和 6%存在 SGS 和支气管内疾病，且可能在其他器官无疾病活动的情况下发生。动态呼气胸部 CT 可能是 GPA 大气道受累的一种潜在非侵入性筛查试验。
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