BACKGROUND Bronchiectasis is a heterogeneous disease depending on etiology. It represents the most frequent non-infectious pulmonary complication of primary immunodeficiencies (PID). We investigated whether bronchiectasis associated with PID had a distinct course in comparison to bronchiectasis of other causes. METHODS Retrospective single-center study of adult patients diagnosed with non-cystic fibrosis bronchiectasis with more than 5 years of follow-up and at least 4 pulmonary functional tests available at one year apart. They were divided into three groups: PID- related bronchiectasis, idiopathic/post infectious-related bronchiectasis and other causes of bronchiectasis. Respiratory functional data and clinical outcomes were compared. RESULTS Of 329 patients with bronchiectasis diagnosed in Foch Hospital (Suresnes, France), 98 patients fulfilled the selected criteria (20 PID-related cases, 39 idiopathic or post-infectious cases, and 39 cases with other causes). Median time of follow-up was 9.5 years. Groups were similar concerning initial characteristics (female 70.4%, never smokers 59.2%, mild severity bronchiectasis according to the FACED score and median FEV1 at diagnosis 73.5% predicted values [Q1-Q3: 53.75-90.5]), except PID patients who were younger (median age of 51.5 vs 62 years, p = 0.02). Eighty-five percent of PID patients received immunoglobulin substitution (median trough level was measured at 10.5 g/dl [10;10.92]). Global median FEV1 annual decline was 25.03 ml/year [8.16;43.9] and 19.82 ml/year [16.08;48.02] in the PID patients group. Forty-five percent of patients had bacterial colonization, pneumoniae occurred in 56% of patients and median exacerbation annual rate was 0.8 [0.3-1.4]. Hemoptysis occurred in 31.6% of patients. Global mortality rate was 11.2%. We did not record any significant difference for all clinical and functional outcomes between patients with PID and other etiologies. The median decline in FEV1 was similar in the three groups. CONCLUSIONS The course of PID-related bronchiectasis was similar to bronchiectasis of other causes. Provided that patients receive immunoglobulin replacement, the course of PID-related bronchiectasis seems to be independent of the underlying immune disorder.

中文翻译：

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成人原发性免疫缺陷相关性支气管扩张：在法国参考中心与其他病因的支气管扩张比较。

背景技术取决于病因，支气管扩张是一种异质性疾病。它代表了原发性免疫缺陷（PID）的最常见的非感染性肺部并发症。我们调查了与其他原因的支气管扩张相比，与PID相关的支气管扩张是否具有不同的过程。方法回顾性单中心研究对诊断为非囊性纤维化支气管扩张的成年患者进行了5年以上的随访，并且至少相隔一年进行了4次肺功能检查。他们分为三类：PID相关性支气管扩张，特发性/感染后相关性支气管扩张和其他原因的支气管扩张。比较了呼吸功能数据和临床结局。结果在Foch医院（法国Suresnes）诊断出的329例支气管扩张患者中，98名患者符合所选标准（20例PID相关病例，39例特发性或感染后病例以及39例其他原因引起的病例）。随访的中位时间为9.5年。各组在初始特征方面相似（女性为70.4％，从不吸烟者为59.2％，根据FACED评分为轻度严重支气管扩张，诊断时的中值FEV1为预测值的73.5％[Q1-Q3：53.75-90.5]），但较年轻的PID患者除外（中位数年龄为51.5 vs 62岁，p = 0.02）。85％的PID患者接受了免疫球蛋白替代（中谷水平测得为10.5 g / dl [10; 10.92]）。在PID患者组中，FEV1的全球中位数年下降分别为25.03 ml /年[8.16; 43.9]和19.82 ml /年[16.08; 48.02]。45％的患者出现细菌定植，肺炎发生在56％的患者中，中位急性发作年率为0.8 [0.3-1.4]。咯血发生在31.6％的患者中。全球死亡率为11.2％。对于PID和其他病因的患者，我们在所有临床和功能结局方面均未发现任何显着差异。在这三组中，FEV1的中位数下降幅度相似。结论PID相关性支气管扩张的过程与其他原因的支气管扩张相似。如果患者接受免疫球蛋白替代，PID相关性支气管扩张的病程似乎与潜在的免疫疾病无关。对于PID和其他病因的患者，我们在所有临床和功能结局方面均未发现任何显着差异。在这三组中，FEV1的中位数下降幅度相似。结论PID相关性支气管扩张的过程与其他原因的支气管扩张相似。如果患者接受免疫球蛋白替代，PID相关性支气管扩张的病程似乎与潜在的免疫疾病无关。对于PID和其他病因的患者，我们在所有临床和功能结局方面均未发现任何显着差异。在这三组中，FEV1的中位数下降幅度相似。结论PID相关性支气管扩张的过程与其他原因的支气管扩张相似。如果患者接受免疫球蛋白替代，PID相关性支气管扩张的病程似乎与潜在的免疫疾病无关。