Bronchiectasis is a well-recognised complication of primary antibody deficiency (PAD) syndromes. Previous data suggest that mortality in common variable immune deficiency (CVID) is not associated with isolated bronchiectasis. A retrospective analysis of patients with CVID and specific antibody deficiency in two tertiary referral centres with lung disease was conducted. Severity of bronchiectasis at presentation was associated with mortality. Lower FEV1, colonisation with Pseudomonas aeruginosa and a diagnosis of COPD were also associated with mortality. Bronchiectasis is an important driver of mortality in patients with PAD syndromes.

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支气管扩张严重程度与原发抗体缺乏患者的预后相关

支气管扩张是公认的原发性抗体缺乏 (PAD) 综合征并发症。先前的数据表明，常见可变免疫缺陷 (CVID) 的死亡率与孤立的支气管扩张症无关。对两个肺病三级转诊中心的 CVID 和特异性抗体缺乏患者进行了回顾性分析。就诊时支气管扩张的严重程度与死亡率相关。较低的 FEV1、铜绿假单胞菌定植和 COPD 诊断也与死亡率相关。支气管扩张是 PAD 综合征患者死亡率的重要驱动因素。