在吸烟人群中，α1-抗胰蛋白酶缺乏症和吸烟相关性慢性阻塞性肺疾病的 CT 表现具有相似性。

Similarities in the Computed Tomography Appearance in α1-Antitrypsin Deficiency and Smoking-Related Chronic Obstructive Pulmonary Disease in a Smoking Collective.

机构信息

Department of Diagnostic and Interventional Radiology, University Hospital of Heidelberg, Heidelberg, Germany.

Translational Lung Research Center (TLRC) Heidelberg, Member of the German Center for Lung Research (DZL), Heidelberg, Germany.

出版信息

Respiration. 2018;96(3):231-239. doi: 10.1159/000489177. Epub 2018 Jun 25.

DOI:10.1159/000489177

PMID:29940576

Abstract

BACKGROUND

Emphysematous destruction of lung parenchyma visible in computed tomography (CT) can be attributed to chronic obstructive pulmonary disease (COPD) or to α1-antitrypsin deficiency (AATD).

OBJECTIVES

We evaluated if visual semiquantitative phenotyping of CT data helps identifying individuals with AATD in a group of smokers with severe emphysema and airflow limitation.

METHOD

n = 14 patients with AATD and n = 15 with COPD and a minimum of 10 pack years underwent CT, clinical assessment, and full-body plethysmography. The extent and type of emphysema as well as large and small airway changes were rated semiquantitatively for each lobe using a standardized previously published scoring system. Lastly, a final diagnosis for each patient was proposed.

RESULTS

AATD had a significantly lower mean emphysema score than COPD, with 8.9 ± 3.4 versus 11.9 ± 3.2 (p < 0.001), respectively. Within both groups, there was significantly more emphysema in the lower lobes (p < 0.05-0.001). The COPD group showed an upper- and middle-lobe predominance of emphysema distribution when compared to the AATD group (p < 0.001). Centrilobular (CLE) and panlobular (PLE) emphysema patterns showed a uniform distribution within both groups, with a CLE predominance in the upper lung and a PLE predominance in the lower lung regions. AATD and COPD both showed significantly more airway changes in lower lobes compared to upper lobes (p = 0.05-0.001), without significant differences between both groups.

CONCLUSION

The typical emphysema distribution patterns seen on CT traditionally assigned to AATD and COPD were of little use in discriminating both entities. Also, airway changes could not contribute to a more precise differentiation. We conclude that a concise standardized phenotyping-driven approach to chest CT in emphysema is not sufficient to identify patients with AATD in a cohort of smokers with advanced emphysema.

摘要

背景

在计算机断层扫描（CT）中可见的肺气肿性肺实质破坏可归因于慢性阻塞性肺疾病（COPD）或α1-抗胰蛋白酶缺乏症（AATD）。

目的

我们评估了在一组严重肺气肿和气流受限的吸烟者中，通过 CT 数据的视觉半定量表型分析是否有助于识别 AATD 个体。

方法

n = 14 例 AATD 患者和 n = 15 例 COPD 患者，且吸烟至少 10 包年，进行 CT、临床评估和全身肺量测定。使用以前发表的标准化评分系统，对每个肺叶的肺气肿程度和类型以及大气道和小气道变化进行半定量评分。最后，对每位患者提出最终诊断。

结果

AATD 的平均肺气肿评分明显低于 COPD，分别为 8.9 ± 3.4 与 11.9 ± 3.2（p < 0.001）。在两组中，下叶的肺气肿均明显更多（p < 0.05-0.001）。与 AATD 组相比，COPD 组的肺气肿分布具有中上叶优势（p < 0.001）。两组均表现为小叶中央型（CLE）和全小叶型（PLE）肺气肿模式，上肺以 CLE 为主，下肺以 PLE 为主。与上叶相比，AATD 和 COPD 在下叶均表现出明显更多的气道变化（p = 0.05-0.001），但两组之间无显著差异。