Department of Internal Medicine V, Pulmonology, Allergology, Intensive Care Medicine, Saarland University Hospital, 66424, Homburg, Germany.
Institute for Biostatistics, Hannover Medical School, 30625, Hannover, Germany.
Respir Res. 2017 Sep 15;18(1):171. doi: 10.1186/s12931-017-0655-1.
Alpha-1-antitrypsin deficiency (AATD) is a rare inherited condition caused by mutations of the SERPINA1 gene that is associated with the development of a COPD like lung disease. The comorbidities in patients with AATD-related lung diseases are not well defined. The aim of this study was to analyze the clinical phenotype of AATD patients within the German COPD cohort study COSYCONET ("COPD and SYstemic consequences-COmorbidities NETwork") cohort focusing on the distribution of comorbidities.
The data from 2645 COSYCONET patients, including 139 AATD patients (110 with and 29 without augmentation therapy), were analyzed by descriptive statistics and regression analyses. We found significantly lower prevalence of cardiovascular comorbidities in AATD patients as compared to non-AATD COPD patients. After correction for age, pack years, body mass index, and sex, the differences were still significant for coronary artery disease (p = 0.002) and the prevalence of peripheral artery disease as determined by an ankle-brachial-index <= 0.9 (p = 0.035). Also the distribution of other comorbidities such as bronchiectasis differed between AATD and non-deficient COPD.
AATD is associated with a lower prevalence of cardiovascular disease, the underlying mechanisms need further investigation.
α-1-抗胰蛋白酶缺乏症（AATD）是一种由 SERPINA1 基因突变引起的罕见遗传性疾病，与 COPD 样肺部疾病的发展有关。AATD 相关肺部疾病患者的合并症尚不清楚。本研究的目的是分析德国 COPD 队列研究 COSYCONET（“COPD 和系统性后果-合并症网络”）队列中 AATD 患者的临床表型，重点分析合并症的分布。
对包括 139 名 AATD 患者（110 名接受过和 29 名未接受过补充治疗）在内的 2645 名 COSYCONET 患者的数据进行了描述性统计和回归分析。与非 AATD COPD 患者相比，AATD 患者的心血管合并症患病率明显较低。在校正年龄、吸烟包年数、体重指数和性别后，冠心病（p=0.002）和踝肱指数≤0.9（p=0.035）的外周动脉疾病患病率差异仍有统计学意义。AATD 和非缺乏型 COPD 之间的其他合并症（如支气管扩张症）的分布也存在差异。
AATD 与心血管疾病的患病率较低有关，其潜在机制需要进一步研究。
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