Division of Allergy and Immunology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Division of Allergy-Immunology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
J Clin Immunol. 2019 Aug;39(6):569-576. doi: 10.1007/s10875-019-00657-w. Epub 2019 Jun 27.
Chronic lung disease is the most common cause of morbidity and mortality in patients with common variable immunodeficiency (CVID). While biomarkers exist to predict non-infectious complications, the unique features that define CVID patients with chronic lung disease are not well understood.
We analyzed data from CVID patients from the retrospective USIDNET (United States Immunodeficiency Network) patient database. Patients were categorized into 3 phenotypes for comparison: (1) CVID without chronic lung disease, (2) CVID with bronchiectasis only, and (3) CVID with interstitial lung disease (ILD) with or without bronchiectasis. Among these groups, differences were assessed in demographics, comorbidities, infections, treatments, and peripheral blood immune measures. We analyzed 1518 CVID patients which included 1233 (81.2%) without chronic lung disease, 147 (9.7%) with bronchiectasis only, and 138 (9.1%) with interstitial lung disease.
Patients with ILD had lower CD3 cell counts (P = .001), CD4 cell counts (P < .05), and CD8 cell counts (P < .001) compared with patients without lung disease. Additionally, there was significantly more CVID patients with ILD with pneumonia (P < .001), herpes viruses (P = .01) and fungal infections (P < .001) compared with patients with CVID without chronic lung disease.
This analysis suggests that patients with chronic lung disease may be more likely to have lower peripheral T cell counts and complications of those defects compared with CVID patients without chronic lung disease.
慢性肺部疾病是普通变异性免疫缺陷（CVID）患者发病率和死亡率的最常见原因。虽然存在预测非传染性并发症的生物标志物，但定义患有慢性肺部疾病的 CVID 患者的独特特征尚不清楚。
我们分析了来自美国免疫缺陷网络（USIDNET）回顾性患者数据库的 CVID 患者的数据。患者分为 3 种表型进行比较：（1）无慢性肺部疾病的 CVID；（2）仅支气管扩张的 CVID；（3）有或无支气管扩张的间质性肺病（ILD）的 CVID。在这些组中，评估了人口统计学、合并症、感染、治疗和外周血免疫措施的差异。我们分析了 1518 例 CVID 患者，其中 1233 例（81.2%）无慢性肺部疾病，147 例（9.7%）仅支气管扩张，138 例（9.1%）间质性肺病。
与无肺部疾病的患者相比，ILD 患者的 CD3 细胞计数（P = .001）、CD4 细胞计数（P < .05）和 CD8 细胞计数（P < .001）较低。此外，与无慢性肺部疾病的 CVID 患者相比，ILD 患者患有肺炎（P < .001）、疱疹病毒（P = .01）和真菌感染（P < .001）的 CVID 患者明显更多。
这项分析表明，与无慢性肺部疾病的 CVID 患者相比，患有慢性肺部疾病的患者可能更有可能具有较低的外周 T 细胞计数和这些缺陷的并发症。
