In this review paper, we discuss the characteristics that define severe bronchiectasis and which may lead to deterioration of noncystic fibrosis bronchiectasis. These characteristics were used to establish the current severity scores: bronchiectasis severity index (BSI), FACED, and E-FACED (exacerbation frequency, forced expiratory volume in 1 second, age, colonization, extension and dyspnea score). They can be used to predict mortality, exacerbation rate, hospital admission, and quality of life. Furthermore, there are different treatable traits that contribute to severe bronchiectasis and clinical deterioration. When present, they can be a target of the treatment to stabilize bronchiectasis.

One of the first steps in treatment management of bronchiectasis is evaluation of compliance to already prescribed therapy. Several factors can contribute to treatment adherence, but to date no real interventions have been published to ameliorate this phenomenon. In the second step, treatment in deteriorating patients with bronchiectasis should be guided by the predominant symptoms, for example, cough, sputum, difficulty expectoration, exacerbation rate, or physical impairment. In the third step, we evaluate treatable traits that could influence disease severity in the deteriorating patient. Finally, in patients who are difficult to treat despite maximum medical treatment, eligibility for surgery (when disease is localized), should be considered. In case of end-stage disease, the evaluation for lung transplantation should be performed. Noninvasive ventilation can serve as a bridge to lung transplantation in patients with respiratory failure.

在这篇综述文章中，我们讨论了定义严重支气管扩张的特征以及可能导致非囊性纤维化支气管扩张恶化的特征。这些特征用于建立当前的严重程度评分：支气管扩张严重程度指数 (BSI)、FACED 和 E-FACED（恶化频率、1 秒内用力呼气量、年龄、定植、延伸和呼吸困难评分）。它们可用于预测死亡率、恶化率、住院率和生活质量。此外，有不同的可治疗特征会导致严重的支气管扩张和临床恶化。如果存在，它们可以成为稳定支气管扩张的治疗目标。

支气管扩张治疗管理的第一步是评估对已开处方的治疗的依从性。有几个因素可以促进治疗依从性，但迄今为止，还没有发表真正的干预措施来改善这种现象。第二步，对恶化的支气管扩张患者的治疗应以咳嗽、咳痰、咳痰困难、加重率或身体损伤等主要症状为指导。第三步，我们评估可能影响恶化患者疾病严重程度的可治疗特征。最后，对于尽管进行了最大限度的药物治疗仍难以治疗的患者，应考虑是否适合手术（当疾病局限于局部时）。如果是终末期疾病，应进行肺移植评估。