De Renzis Chiara, Gambier Renato Finco, Gigante Antonietta, Deiana Carla Maria, Lagnese Gianluca, Gatti Lorenzo, Garzi Giulia, Costanzo Giulia, Pagnozzi Chiara, Nicola Stefania, Brussino Luisa, Spadaro Giuseppe, Rattazzi Marcello, Firinu Davide, Cinetto Francesco, Milito Cinzia
Department of Translational and Precision Medicine, "Sapienza" University of Rome, Rome, Italy.
Rare Disease Referral Centre, Internal Medicine I, Department of Medicine, Cà Foncello Hospital, @AULSS2 Marca Trevigiana, DIMED - University of Padua, Treviso, Italy.
J Clin Immunol. 2025 May 23;45(1):97. doi: 10.1007/s10875-025-01890-2.
There are few reports of renal involvement in Common Variable Immunodeficiencies (CVID) and, when present, is due to infections, inflammation, or treatments. The aim of this study was evaluating the prevalence of chronic kidney disease (CKD) and to identify CVID-related clinical, laboratory and therapeutic features inducing it.
A multicenter observational retrospective study on 367 adult CVID patients from five Italian Referral Centers for Primary Immunodeficiency.
CKD was identified in 23 (6.27%) patients that were older (p < 0.001), had arterial hypertension (p < 0.001), diabetes (p = 0.002), dyslipidemia (p = 0.002), presented different ultrasound abnormalities (p < 0.001) and received predominantly intravenous immunoglobulins (IVIG) (p = 0.016). Regarding CVID infectious and non-infectious manifestations, CKD patients presented a higher frequency of COPD (p = 0.008). In the CKD group, the median absolute count of total lymphocytes (p = 0.015), the percentage of total B (p = 0.028) and transitional B cells (p = 0.008) were lower. By binomial logistic regression analysis adjusted for age, CKD patients tend to develop autoimmune cytopenia, had lower B cells percentage, increased Neutrophil-to-lymphocyte ratio and received more frequently trimethoprim-sulfamethoxazole antibiotic prophylaxis. By multivariate analysis, only autoimmune cytopenia was independently associated with CKD.
The prevalence of CKD in CVID is due to aging, age-related comorbidities, disease-related immune dysregulation and inflammation. Our results suggest evaluating renal function in all CVID patients, and mostly in those with a higher "inflammatory" burden.
关于常见可变免疫缺陷（CVID）合并肾脏受累的报道较少，若出现肾脏受累，多归因于感染、炎症或治疗。本研究旨在评估慢性肾脏病（CKD）的患病率，并确定导致其发生的与CVID相关的临床、实验室及治疗特征。
对来自意大利五个原发性免疫缺陷转诊中心的367例成年CVID患者进行多中心观察性回顾研究。
在23例（6.27%）患者中发现了CKD，这些患者年龄较大（p<0.001），患有动脉高血压（p<0.001）、糖尿病（p=0.002）、血脂异常（p=0.002），出现不同的超声异常（p<0.001），且主要接受静脉注射免疫球蛋白（IVIG）治疗（p=0.016）。关于CVID的感染性和非感染性表现，CKD患者慢性阻塞性肺疾病（COPD）的发生率更高（p=0.008）。在CKD组中，总淋巴细胞绝对计数中位数（p=0.015）、总B细胞百分比（p=0.028）和过渡性B细胞百分比（p=0.008）较低。经年龄校正的二项式逻辑回归分析显示，CKD患者更容易发生自身免疫性血细胞减少，B细胞百分比更低，中性粒细胞与淋巴细胞比值升高，且更频繁地接受甲氧苄啶-磺胺甲恶唑抗生素预防。多因素分析显示，只有自身免疫性血细胞减少与CKD独立相关。
CVID患者中CKD的患病率归因于衰老、与年龄相关的合并症、疾病相关的免疫失调和炎症。我们的结果提示，应对所有CVID患者，尤其是那些“炎症”负担较高的患者进行肾功能评估。
