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体液原发性免疫缺陷的高分辨率计算机断层扫描结果及其与肺功能测试的相关性。

High-resolution computed tomography findings in humoral primary immunodeficiencies and correlation with pulmonary function tests.

作者信息

Cereser Lorenzo, De Carli Marco, d'Angelo Paola, Zanelli Elisa, Zuiani Chiara, Girometti Rossano

机构信息

Institute of Radiology, Department of Medicine, University of Udine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy.

Second Unit of Internal Medicine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy.

出版信息

World J Radiol. 2018 Nov 28;10(11):172-183. doi: 10.4329/wjr.v10.i11.172.

Abstract

AIM

To compare high-resolution computed tomography (HRCT) findings between humoral primary immunodeficiencies (hPIDs) subtypes; to correlate these findings to pulmonary function tests (PFTs).

METHODS

We retrospectively identified 52 consecutive adult patients with hPIDs who underwent 64-row HRCT and PFTs at the time of diagnosis. On a per-patient basis, an experienced radiologist recorded airway abnormalities (bronchiectasis, airway wall thickening, mucus plugging, tree-in-bud, and air-trapping) and parenchymal-interstitial abnormalities (consolidations, ground-glass opacities, linear and/or irregular opacities, nodules, and bullae/cysts) found on HRCT. The chi-square test was performed to compare the prevalence of each abnormality among patients with different subtypes of hPIDs. Overall logistic regression analysis was performed to assess whether HRCT findings predicted obstructive and/or restrictive PFTs results (absent-to-mild moderate-to-severe).

RESULTS

Thirty-eight of the 52 patients with hPIDs showed common variable immunodeficiency disorders (CVID), while the remaining 14 had CVID-like conditions (., 11 had isolated IgG subclass deficiencies and 3 had selective IgA deficiencies). The prevalence of most HRCT abnormalities was not significantly different between CVID and CVID-like patients ( > 0.05), except for linear and/or irregular opacities (prevalence of 31.6% in the CVID group and 0 in the CVID-like group; = 0.0427). Airway wall thickening was the most frequent HRCT abnormality found in both CVID and CVID-like patients (71% of cases in both groups). The presence of tree-in-bud abnormalities was an independent predictor of moderate-to-severe obstructive defects at PFTs (Odds Ratio, OR, of 18.75, < 0.05), while the presence of linear and/or irregular opacities was an independent predictor of restrictive defects at PFTs (OR = 13.00; < 0.05).

CONCLUSION

CVID and CVID-like patients showed similar HRCT findings. Tree-in-bud and linear and/or irregular opacities predicted higher risks of, respectively, obstructive and restrictive defects at PFTs.

摘要

目的

比较体液原发性免疫缺陷(hPIDs)各亚型之间的高分辨率计算机断层扫描(HRCT)结果;将这些结果与肺功能测试(PFTs)相关联。

方法

我们回顾性纳入了52例连续的成年hPIDs患者,这些患者在诊断时接受了64排HRCT和PFTs检查。由一位经验丰富的放射科医生逐例记录HRCT上发现的气道异常(支气管扩张、气道壁增厚、黏液嵌塞、树芽征和气陷)以及实质-间质异常(实变、磨玻璃影、线性和/或不规则影、结节以及肺大泡/囊肿)。采用卡方检验比较不同hPIDs亚型患者中各异常的发生率。进行总体逻辑回归分析以评估HRCT结果是否能预测阻塞性和/或限制性PFTs结果(无至轻度 与 中度至重度)。

结果

52例hPIDs患者中,38例表现为常见变异型免疫缺陷病(CVID),其余(14例有类似CVID的情况(例如,11例有孤立的IgG亚类缺陷,3例有选择性IgA缺陷)。除线性和/或不规则影外,CVID和类似CVID患者中大多数HRCT异常的发生率无显著差异(P>0.05)(CVID组中线性和/或不规则影的发生率为31.6%,类似CVID组中为0;P = 0.0427)。气道壁增厚是CVID和类似CVID患者中最常见的HRCT异常(两组病例中均为71%)。树芽征异常的存在是PFTs中中度至重度阻塞性缺陷的独立预测因素(优势比,OR,为18.75,P<0.05),而线性和/或不规则影的存在是PFTs中限制性缺陷的独立预测因素(OR = 13.00;P<0.05)。

结论

CVID和类似CVID患者表现出相似的HRCT结果。树芽征以及线性和/或不规则影分别预测了PFTs中阻塞性和限制性缺陷的较高风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5ac5/6288673/d6c4c47d8e14/WJR-10-172-g001.jpg

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