OBJECTIVE: What is the frequency of PCD among adults with bronchiectasis; how do people with PCD differ from those with other etiologies; and which clinical characteristics are independently associated with PCD?
METHODS: We investigated the proportion of PCD among the participants of the Prospective German Non-CF-Bronchiectasis Registry (PROGNOSIS) study; applied multiple imputation to account for missing data in 64 (FEV1), 58 (breathlessness), 26 (pulmonary exacerbations), and two patients (BMI), respectively; and identified predictive variables from baseline data using multivariate logistic regression analysis.
RESULTS: We consecutively recruited 1,000 patients from 38 centers across all levels of the German health care system. Overall, PCD was the fifth most common etiology of bronchiectasis in 87 patients (9%) after idiopathic, postinfective, COPD, and asthma. People with PCD showed a distinct clinical phenotype. In multivariate regression analysis, the chance of PCD being the etiology of bronchiectasis increased with the presence of upper airway disease (chronic rhinosinusitis and/or nasal polyps; adjusted OR [aOR], 6.3; 95% CI, 3.3-11.9; P < .001), age < 53 years (aOR, 5.3; 95% CI, 2.7-10.4; P < .001), radiologic involvement of any middle and lower lobe (aOR, 3.7; 95% CI, 1.3-10.8; P = .016), duration of bronchiectasis > 15 years (aOR, 3.6; 95% CI, 1.9-6.9; P < .001), and a history of Pseudomonas aeruginosa isolation from respiratory specimen (aOR, 2.4; 95% CI, 1.3-4.5; P = .007).
CONCLUSIONS: Within our nationally representative cohort, PCD was a common etiology of bronchiectasis. We identified few easy-to-assess phenotypic features, which may promote awareness for PCD among adults with bronchiectasis.
BACKGROUND: ClinicalTrials.gov; No.: NCT02574143; URL: www.
RESULTS: gov.
■患有支气管扩张症的成年人中PCD的频率是多少?PCD患者与其他病因的患者有何不同?哪些临床特征与PCD独立相关?
方法:我们调查了德国支气管扩张注册方案参与者中PCD的比例,应用多重插补来解决64(FEV1)中的缺失数据,58(呼吸困难),26(肺加重),和2名受试者(BMI),分别,并使用多变量逻辑回归分析从基线数据中确定预测变量。
结果:我们从德国各级医疗保健系统的38个中心连续招募了1,000名患者。总的来说,PCD是继特发性、感染后,COPD,和哮喘。PCD患者表现出明显的临床表型。在多元回归分析中,PCD是支气管扩张的病因的机会随着上呼吸道疾病的存在而增加(慢性鼻-鼻窦炎和/或鼻息肉;aOR,6.3;95%CI3.3-11.9;P<.001);年龄<53岁(aOR,5.3;95%CI2.7-10.4;P<.001);任何中下叶的放射学受累(aOR,3.7;95%CI1.3-10.8;P=0.016);支气管扩张持续时间>15年(aOR,3.6;95%CI1.9-6.9;P<.001);以及从呼吸道标本中分离铜绿假单胞菌的病史(aOR,2.4;95%CI1.3-4.5;P=0.007)。
结论:在我们具有全国代表性的队列中,PCD是支气管扩张的常见病因。我们发现了一些易于评估的表型特征,这可能会提高支气管扩张成年人对PCD的认识。