BACKGROUND The long-term clinical course of asthma in patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA) remains unclear. We aimed to characterize long-term asthma in EGPA and to identify baseline predictors of long-term asthma severity. METHODS Retrospective cohort study of patients who fulfilled standardized criteria for EGPA that were followed in a single referral center between 1990-2017. Baseline and 3 (±1) years of follow-up clinical, laboratory and pulmonary function data were analyzed. RESULTS Eighty-nine patients with EGPA, and a documented asthma assessment at baseline and at 3 years from diagnosis were included. Severe/uncontrolled asthma was observed in 42.7% of patients at diagnosis and was associated with previous history of respiratory allergy (p<0.01), elevated serum total IgE levels (p<0.05), increased use of high dose inhaled (ICS; p<0.05) and oral corticosteroids (OCS; p<0.001) for respiratory symptoms the year before the diagnosis of EGPA. During follow-up, an improvement or worsening in asthma severity was noted in 12.3% and 10.1% of patients, respectively. Severe/uncontrolled asthma was present in 40.5% of patients at 3 years, and was associated with increased airway resistance on pulmonary function testing (p<0.05). Long-term PFTs did not improve during long-term follow-up regardless of ICS or OCS therapy. Using multivariate binary logistic regressions, severe rhinosinusitis (p=0.038), pulmonary infiltrates (p=0.011), overweight (BMI>25kg/m2; p=0.041) and severe/uncontrolled asthma at vasculitis diagnosis (p<0.001) independently predicted severe/uncontrolled asthma at the 3-year endpoint. CONCLUSION In asthmatic patients with EGPA, long-term severe/uncontrolled asthma is associated with baseline pulmonary and ENT manifestations, but not with clear-cut vasculitic features.

中文翻译：

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嗜酸性肉芽肿伴多血管炎：长期哮喘严重程度的临床预测因素。

背景 嗜酸性肉芽肿伴多血管炎 (EGPA) 患者哮喘的长期临床病程仍不清楚。我们旨在表征 EGPA 中的长期哮喘，并确定长期哮喘严重程度的基线预测因素。方法 回顾性队列研究符合 EGPA 标准化标准的患者，这些患者在 1990 年至 2017 年期间在单一转诊中心接受随访。分析了基线和 3 (±1) 年随访的临床、实验室和肺功能数据。结果 纳入了 89 名 EGPA 患者，并在基线和诊断后 3 年进行了有记录的哮喘评估。42.7% 的患者在诊断时观察到严重/未控制的哮喘，并且与既往呼吸道过敏史 (p<0.01)、血清总 IgE 水平升高 (p<0.05)、在诊断 EGPA 前一年，增加使用高剂量吸入（ICS；p<0.05）和口服皮质类固醇（OCS；p<0.001）治疗呼吸道症状。在随访期间，分别有 12.3% 和 10.1% 的患者发现哮喘严重程度有所改善或恶化。3 年时 40.5% 的患者出现严重/未控制的哮喘，并且与肺功能测试中气道阻力增加有关（p<0.05）。无论 ICS 或 OCS 治疗如何，长期随访期间长期 PFT 均未改善。使用多元二元逻辑回归，重度鼻窦炎 (p=0.038)、肺部浸润 (p=0.011)、超重 (BMI>25kg/m2；p=0.041) 和诊断为血管炎时的重度/不受控制的哮喘 (p<0.001) 独立预测重度/ 3 年终点未控制的哮喘。