Abstract：
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease, predisposing to an increased risk of infection. A complete picture of these infections is lacking.
Describe the characteristics and clinical outcomes of patients diagnosed with aPAP, and to identify risk factors associated with opportunistic infections.
We conducted a retrospective cohort including all patients diagnosed with aPAP between 2008 and 2018 in France and Belgium. Data were collected using a standardised questionnaire including demographics, comorbidities, imaging features, outcomes and microbiological data.
We included 104 patients, 2/3 were men and median age at diagnosis was 45 years. With a median follow-up of 3.4 years (IQR 1.7-6.6 years), 60 patients (58%), developed at least one infection, including 23 (22%) with opportunistic infections. Nocardia spp was the main pathogen identified (n=10). Thirty-five (34%) patients were hospitalised due to infection. In univariate analysis, male gender was associated with opportunistic infections (p=0.04, OR=3.88; 95% CI (1.02 to 22.06)). Anti-granulocyte macrophage colony-stimulating factor antibody titre at diagnosis was significantly higher among patients who developed nocardiosis (1058 (316-1591) vs 580 (200-1190), p=0.01). Nine patients had died (9%), but only one death was related to infection.
Patients with aPAP often presented with opportunistic infections, especially nocardiosis, which highlights the importance of systematic search for slow-growing bacteria in bronchoalveolar lavage or whole lung lavage.
Describe the characteristics and clinical outcomes of patients diagnosed with aPAP, and to identify risk factors associated with opportunistic infections.
We conducted a retrospective cohort including all patients diagnosed with aPAP between 2008 and 2018 in France and Belgium. Data were collected using a standardised questionnaire including demographics, comorbidities, imaging features, outcomes and microbiological data.
We included 104 patients, 2/3 were men and median age at diagnosis was 45 years. With a median follow-up of 3.4 years (IQR 1.7-6.6 years), 60 patients (58%), developed at least one infection, including 23 (22%) with opportunistic infections. Nocardia spp was the main pathogen identified (n=10). Thirty-five (34%) patients were hospitalised due to infection. In univariate analysis, male gender was associated with opportunistic infections (p=0.04, OR=3.88; 95% CI (1.02 to 22.06)). Anti-granulocyte macrophage colony-stimulating factor antibody titre at diagnosis was significantly higher among patients who developed nocardiosis (1058 (316-1591) vs 580 (200-1190), p=0.01). Nine patients had died (9%), but only one death was related to infection.
Patients with aPAP often presented with opportunistic infections, especially nocardiosis, which highlights the importance of systematic search for slow-growing bacteria in bronchoalveolar lavage or whole lung lavage.
摘要：
背景：自身免疫性肺泡蛋白沉积症（aPAP）是一种罕见的疾病，易感感染风险增加。缺乏这些感染的完整图片。
目的：描述诊断为aPAP的患者的特征和临床结局，并确定与机会性感染相关的危险因素。
方法：我们进行了一项回顾性队列研究，包括2008年至2018年在法国和比利时诊断为aPAP的所有患者。数据是使用标准化问卷收集的，包括人口统计，合并症,成像特征,结果和微生物数据。
结果：我们纳入了104例患者，2/3为男性，诊断时的中位年龄为45岁。中位随访时间为3.4年（IQR1.7-6.6年），60名患者（58%），至少发生了一次感染，包括23例（22%）机会性感染。诺卡氏菌属是确定的主要病原体（n=10）。35例（34%）患者因感染住院。在单变量分析中，男性与机会性感染相关(p=0.04,OR=3.88;95%CI(1.02~22.06)).诊断时的抗粒细胞巨噬细胞集落刺激因子抗体滴度在发生诺卡病的患者中明显更高（1058（316-1591）vs580（200-1190），p=0.01)。9名患者死亡（9%），但只有一人死亡与感染有关.
结论：aPAP患者常出现机会性感染，尤其是诺卡心症,这突出了在支气管肺泡灌洗或全肺灌洗中系统寻找生长缓慢的细菌的重要性。
目的：描述诊断为aPAP的患者的特征和临床结局，并确定与机会性感染相关的危险因素。
方法：我们进行了一项回顾性队列研究，包括2008年至2018年在法国和比利时诊断为aPAP的所有患者。数据是使用标准化问卷收集的，包括人口统计，合并症,成像特征,结果和微生物数据。
结果：我们纳入了104例患者，2/3为男性，诊断时的中位年龄为45岁。中位随访时间为3.4年（IQR1.7-6.6年），60名患者（58%），至少发生了一次感染，包括23例（22%）机会性感染。诺卡氏菌属是确定的主要病原体（n=10）。35例（34%）患者因感染住院。在单变量分析中，男性与机会性感染相关(p=0.04,OR=3.88;95%CI(1.02~22.06)).诊断时的抗粒细胞巨噬细胞集落刺激因子抗体滴度在发生诺卡病的患者中明显更高（1058（316-1591）vs580（200-1190），p=0.01)。9名患者死亡（9%），但只有一人死亡与感染有关.
结论：aPAP患者常出现机会性感染，尤其是诺卡心症,这突出了在支气管肺泡灌洗或全肺灌洗中系统寻找生长缓慢的细菌的重要性。
