Common variable immunodeficiency disorders (CVID) are multi‐system disorders where target organ damage is mediated by infective, autoimmune and inflammatory processes. Bronchiectasis is probably the most common disabling complication of CVID. The risk factors for bronchiectasis in CVID patients are incompletely understood. The New Zealand CVID study (NZCS) is a nationwide longitudinal observational study of adults, which commenced in 2006. In this analysis, the prevalence and risk factors for bronchiectasis were examined in the NZCS. After informed consent, clinical and demographic data were obtained with an interviewer‐assisted questionnaire. Linked electronic clinical records and laboratory results were also reviewed. Statistical methods were applied to determine if variables such as early‐onset disease, delay in diagnosis and increased numbers of infections were associated with greater risk of bronchiectasis. One hundred and seven adult patients with a diagnosis of CVID are currently enrolled in the NZCS, comprising approximately 70% of patients known to have CVID in New Zealand. Fifty patients (46·7%) had radiologically proven bronchiectasis. This study has shown that patients with compared to those without bronchiectasis have an increased mortality at a younger age. CVID patients with bronchiectasis had a greater number of severe infections consequent to early‐onset disease and delayed diagnosis. Indigenous Māori have a high prevalence of CVID and a much greater burden of bronchiectasis compared to New Zealand Europeans. Diagnostic latency has not improved during the study period. Exposure to large numbers of infections because of early‐onset disease and delayed diagnosis was associated with an increased risk of bronchiectasis. Earlier diagnosis and treatment of CVID may reduce the risk of bronchiectasis and premature death in some patients.

中文翻译：

---

在新西兰常见的可变免疫缺陷疾病队列研究中，支气管扩张与延迟诊断和不良结果有关

常见的可变性免疫缺陷病 (CVID) 是多系统疾病，其中靶器官损伤由感染、自身免疫和炎症过程介导。支气管扩张可能是 CVID 最常见的致残并发症。CVID 患者支气管扩张的危险因素尚不完全清楚。新西兰 CVID 研究 (NZCS) 是一项于 2006 年开始的全国性成人纵向观察研究。在这项分析中，在 NZCS 中检查了支气管扩张的患病率和危险因素。知情同意后，通过访问员辅助问卷获得临床和人口统计数据。还审查了相关的电子临床记录和实验室结果。应用统计方法来确定变量，如早发性疾病、诊断延迟和感染数量增加与支气管扩张的风险增加有关。目前有 107 名诊断为 CVID 的成年患者参加了 NZCS，约占新西兰已知患有 CVID 的患者的 70%。50 名患者 (46·7%) 经放射学证实为支气管扩张。这项研究表明，与没有支气管扩张的患者相比，年轻时的死亡率增加。患有支气管扩张的 CVID 患者由于早发疾病和延误诊断而出现更多的严重感染。与新西兰欧洲人相比，土著毛利人的 CVID 患病率很高，支气管扩张的负担要大得多。在研究期间，诊断潜伏期没有改善。由于早发疾病和延迟诊断而暴露于大量感染与支气管扩张的风险增加有关。CVID的早期诊断和治疗可能会降低一些患者支气管扩张和过早死亡的风险。