PURPOSE Pulmonary manifestations are common in patients with primary immunodeficiency disorders (PIDs) but the prevalence, specific diseases, and their patterns are not well characterized. METHODS We conducted a retrospective analysis of pulmonary diseases reported in the database of the United States Immunodeficiency Network (USIDNET), a program of the Immune Deficiency Foundation. PIDs were categorized into 10 groups and their demographics, pulmonary diagnoses and procedures, infections, prophylaxis regimens, and laboratory findings were analyzed. RESULTS A total of 1937 patients with various PIDs (39.3% of total patients, 49.6% male, average age 37.9 years (SD = 22.4 years)) were noted to have a pulmonary disease comorbidity. Pulmonary diseases were categorized into broad categories: airway (86.8%), parenchymal (18.5%), pleural (4.6%), vascular (4.3%), and other (13.9%) disorders. Common variable immune deficiency (CVID) accounted for almost half of PIDs associated with airway, parenchymal, and other pulmonary disorders. Pulmonary procedures performed in 392 patients were mostly diagnostic (77.3%) or therapeutic (16.3%). These patients were receiving a wide variety of treatments, which included immunoglobulin replacement (82.1%), immunosuppressive (32.2%), anti-inflammatory (12.7%), biologic (9.3%), and cytokine (7.6%)-based therapies. Prophylactic therapy was being given with antibiotics (18.1%), antifungal (3.3%), and antiviral (2.2%) medications, and 7.1% of patients were on long-term oxygen therapy due to advanced lung disease. CONCLUSIONS Pulmonary manifestations are common in individuals with PID, but long-term pulmonary outcomes are not well known in this group of patients. Further longitudinal follow-up will help to define long-term prognosis of respiratory comorbidities and optimal treatment modalities.

中文翻译：

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原发性免疫缺陷病的肺病负担：来自 USIDNET 登记处的数据。

目的 肺部表现在原发性免疫缺陷病 (PID) 患者中很常见，但其患病率、特定疾病及其模式尚未得到很好的表征。方法 我们对美国免疫缺陷网络 (USIDNET) 数据库中报告的肺部疾病进行了回顾性分析，该数据库是免疫缺陷基金会的一个项目。PID 被分为 10 组，并分析了他们的人口统计学、肺部诊断和程序、感染、预防方案和实验室检查结果。结果 共有 1937 名患有各种 PID 的患者（占总患者的 39.3%，男性占 49.6%，平均年龄 37.9 岁（SD = 22.4 岁））被发现患有肺部疾病。肺部疾病分为大类：气道（86.8%）、实质（18.5%）、胸膜（4.6%）、血管 (4.3%) 和其他 (13.9%) 疾病。常见可变免疫缺陷 (CVID) 几乎占与气道、实质和其他肺部疾病相关的 PID 的一半。在 392 名患者中进行的肺部手术主要是诊断性 (77.3%) 或治疗性 (16.3%)。这些患者接受了多种治疗，包括免疫球蛋白替代疗法 (82.1%)、免疫抑制疗法 (32.2%)、抗炎疗法 (12.7%)、生物疗法 (9.3%) 和基于细胞因子 (7.6%) 的疗法。预防性治疗使用抗生素 (18.1%)、抗真菌 (3.3%) 和抗病毒 (2.2%) 药物，7.1% 的患者因晚期肺病接受长期氧疗。结论 PID 患者的肺部表现很常见，但在这组患者中，长期肺部结果并不为人所知。进一步的纵向随访将有助于确定呼吸系统合并症的长期预后和最佳治疗方式。