非囊性纤维化支气管扩张症的多面性。一种聚类分析方法。

The Multiple Faces of Non-Cystic Fibrosis Bronchiectasis. A Cluster Analysis Approach.

机构信息

1 Pneumology Department, Polytechnic and University La Fe Hospital, Valencia, Spain.

2 Bronchiectasis Group, Girona Biomedical Research Institute (IDIBGI), Doctor Trueta University Hospital, Girona, Spain.

出版信息

Ann Am Thorac Soc. 2016 Sep;13(9):1468-75. doi: 10.1513/AnnalsATS.201510-678OC.

DOI:10.1513/AnnalsATS.201510-678OC

PMID:27348199

Abstract

RATIONALE

The clinical presentation and prognosis of non-cystic fibrosis bronchiectasis are both very heterogeneous.

OBJECTIVES

To identify different clinical phenotypes for non-cystic fibrosis bronchiectasis and their impact on prognosis.

METHODS

Using a standardized protocol, we conducted a multicenter observational cohort study at six Spanish centers with patients diagnosed with non-cystic fibrosis bronchiectasis before December 31, 2005, with a 5-year follow-up from the bronchiectasis diagnosis. A cluster analysis was used to classify the patients into homogeneous groups by means of significant variables corresponding to different aspects of bronchiectasis (clinical phenotypes): age, sex, body mass index, smoking habit, dyspnea, macroscopic appearance of sputum, number of exacerbations, chronic colonization with Pseudomonas aeruginosa, FEV1, number of pulmonary lobes affected, idiopathic bronchiectasis, and associated chronic obstructive pulmonary disease. Survival analysis (Kaplan-Meier method and log-rank test) was used to evaluate the comparative survival of the different subgroups.

MEASUREMENTS AND MAIN RESULTS

A total of 468 patients with a mean age of 63 (15.9) years were analyzed. Of these, 58% were females, 39.7% had idiopathic bronchiectasis, and 29.3% presented with chronic Pseudomonas aeruginosa colonization. Cluster analysis showed four clinical phenotypes: (1) younger women with mild disease, (2) older women with mild disease, (3) older patients with severe disease who had frequent exacerbations, and (4) older patients with severe disease who did not have frequent exacerbations. The follow-up period was 54 months, during which there were 95 deaths. Mortality was low in the first and second groups (3.9% and 7.6%, respectively) and high for the third (37%) and fourth (40.8%) groups. The third cluster had a higher proportion of respiratory deaths than the fourth (77.8% vs. 34.4%; P < 0.001).

CONCLUSIONS

Using cluster analysis, it is possible to separate patients with bronchiectasis into distinct clinical phenotypes with different prognoses.

摘要

背景

非囊性纤维化性支气管扩张症的临床表现和预后均具有高度异质性。

目的

确定非囊性纤维化性支气管扩张症的不同临床表型及其对预后的影响。

方法

我们在西班牙的 6 个中心进行了一项多中心观察性队列研究，使用标准化方案，纳入 2005 年 12 月 31 日前确诊的非囊性纤维化性支气管扩张症患者，从支气管扩张症诊断开始进行为期 5 年的随访。通过聚类分析，根据支气管扩张症的不同方面（临床表型）的显著变量将患者分为同质组：年龄、性别、体重指数、吸烟习惯、呼吸困难、痰液宏观外观、加重次数、铜绿假单胞菌慢性定植、FEV1、受影响的肺叶数量、特发性支气管扩张症和相关的慢性阻塞性肺疾病。生存分析（Kaplan-Meier 法和对数秩检验）用于评估不同亚组的比较生存情况。

测量和主要结果

共分析了 468 例平均年龄为 63（15.9）岁的患者。其中，58%为女性，39.7%为特发性支气管扩张症患者，29.3%为慢性铜绿假单胞菌定植患者。聚类分析显示存在 4 种临床表型：（1）年轻女性，病情较轻；（2）老年女性，病情较轻；（3）病情较重且频繁加重的老年患者；（4）病情较重但不频繁加重的老年患者。随访时间为 54 个月，期间有 95 例死亡。第一组和第二组的死亡率较低（分别为 3.9%和 7.6%），而第三组和第四组的死亡率较高（分别为 37%和 40.8%）。第三组的呼吸相关死亡比例高于第四组（77.8%比 34.4%；P<0.001）。