嗜酸性肉芽肿性多血管炎诊断延迟：临床预测因素及早期识别的意义

文献信息

中文摘要

背景： 嗜酸性肉芽肿性多血管炎（EGPA），既往称为Churg-Strauss综合征，是一种罕见的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎，累及多器官。由于表现多样，诊断常具挑战性且被延迟。
目的： 我们旨在评估EGPA患者的诊断延迟情况，并识别与延迟识别相关的临床特征。
方法： 对单中心18岁以上确诊EGPA的成年患者进行回顾性病历审查。收集人口统计学、临床特征、实验室数据、影像学结果、活检结果、治疗及结局等数据。
结果： 共纳入36例患者，从症状出现到诊断的平均时间为4.82年（SD=5.44）。诊断时平均年龄为52.5岁，47.2%为女性。总体而言，哮喘（80.5%）和慢性鼻窦炎（CRS）（80.5%）是最普遍的特征，[待核实：摘要目前报告5.5%的CRS手术；结果部分报告29例中有19例] 在诊断前需要手术。77.8%发现肺结节或浸润，52.8%有鼻息肉，27.8%在诊断前曾接受过生物制剂治疗。在这项探索性分析中，诊断延迟>3年与女性性别（OR 6.7，95% CI 1.66-31.9）、C-ANCA升高（是）相关，后者与诊断延迟的较低几率相关（OR 0.11，95% CI 0.005-0.716，p=0.049），以及既往生物制剂治疗（OR 7.1，95% CI 1.42-54.4）。肾脏受累与诊断延迟的可能性较低相关（p=0.037）。鉴于队列规模小且置信区间宽，这些发现应被解释为产生假设。
结论： 鉴于EGPA的罕见性和多器官表现，诊断EGPA是一项临床挑战。需要高度的临床怀疑以确保早期识别这种罕见疾病，包括全面的系统回顾和适当的实验室检查，特别是在治疗抵抗性哮喘、CRS或嗜酸性粒细胞增多的患者中。

英文摘要

BACKGROUND: Eosinophilic Granulomatosis with Polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a rare antineutrophil cytoplasmic antibody (ANCA) vasculitis, with multiorgan involvement. Due to the variable presentation, diagnosis is often challenging and delayed.
OBJECTIVE: We aimed to assess diagnostic delay among patients with EGPA and identify clinical characteristics associated with delayed recognition.
METHODS: A single-institution retrospective chart review was performed in adult patients over 18 diagnosed with EGPA. Data was collected on demographics, clinical features, laboratory data, imaging results, biopsy results, treatment, and outcomes.
RESULTS: A total of 36 patients were included, with an average time to diagnosis from onset of symptoms of 4.82 years (SD = 5.44). Mean age at diagnosis was 52.5 years and 47.2% were female. Overall, asthma (80.5%) and chronic rhinosinusitis (CRS) (80.5%) were the most prevalent features, and [VERIFY: abstract currently reports 5.5% CRS surgery; Results report 19/29] of those with CRS required surgery prior to diagnosis. Lung nodules or infiltrates were identified in 77.8%, nasal polyps in 52.8%, and 27.8% had received a biologic prior to diagnosis. In this exploratory analysis, diagnostic delay > 3 years was associated with female sex (OR 6.7, 95% CI 1.66-31.9), elevated C-ANCA (yes), which was associated with lower odds of diagnostic delay (OR 0.11, 95% CI 0.005-0.716, p = 0.049), and prior biologic treatment (OR 7.1, 95% CI 1.42-54.4). Renal involvement was associated with a lower likelihood of diagnostic delay (p = 0.037). Given the small cohort and wide confidence intervals, these findings should be interpreted as hypothesis-generating.
CONCLUSION: Diagnosing EGPA presents a clinical challenge given its rarity and multiorgan presentation. A high clinical suspicion is required to ensure earlier recognition of this rare disease, including a thorough review of systems and appropriate laboratory workup, particularly in patients with treatment-resistant asthma, CRS, or eosinophilia.