Bernhard Nikolas, Lepper Philipp M, Vogelmeier Claus, Seibert Martina, Wagenpfeil Stefan, Bals Robert, Fähndrich Sebastian
Department of Internal Medicine V - Pulmonology, Allergology, Intensive Care Medicine, Saarland University Hospital, Homburg.
Department of Medicine, Pulmonary and Critical Care Medicine, University Medical Center Giessen and Marburg, Philipps-University Marburg, Member of the German Center for Lung Research (DZL).
Int J Chron Obstruct Pulmon Dis. 2017 May 12;12:1427-1437. doi: 10.2147/COPD.S130925. eCollection 2017.
Alpha-1-antitrypsin deficiency (AATD) is a rare hereditary disease that is associated with a higher risk to develop chronic obstructive pulmonary disease and liver cirrhosis. Previous cross-sectional studies on AATD individuals have shown a relationship between worse St George's Respiratory Questionnaire (SGRQ) scores and elevated exacerbation rate or high cigarette consumption. There is a lack of longitudinal data on the relationship between the exacerbation rate and worsening of SGRQ during disease. The aim of this study was to provide not only cross-sectional data but also information about the deterioration in quality of life over a follow-up period up to 7 years (median follow-up period of 3.33 years).
We investigated questionnaire-based data of the German AATD registry concerning the relationship between SGRQ and exacerbation frequency, smoking history, forced expiratory volume in 1 second (FEV) and carbon monoxide diffusion capacity (DLCO) first in cross-sectional analysis and later in longitudinal analysis.
Eight hundred sixty-eight individuals with protease inhibitor ZZ (PiZZ) genotype with an average age of 52.6±12.8 years had an SGRQ score of 45.7±20.6. SGRQ significantly correlated with the exacerbation frequency within the last 2 years (=0.464; <0.001), smoking history (=0.233; <0.001), FEV (=-0.436; <0.001), DLCO (=-0.333; <0.001), and patients' age (=0.292; <0.001). Individuals with occupational dust exposure had significantly worse quality of life (<0.001). Mean annual deterioration of SGRQ in all patients with available follow-up data (n=286) was 1.21±4.45 points per year. Univariate and multivariate analysis showed a significant relationship between worsening of SGRQ/year and exacerbation frequency in the follow-up period (=0.144; =0.015).
Worsening of SGRQ is associated with the exacerbation frequency in individuals with PiZZ AATD.
α-1抗胰蛋白酶缺乏症（AATD）是一种罕见的遗传性疾病，与患慢性阻塞性肺疾病和肝硬化的较高风险相关。先前针对AATD患者的横断面研究表明，圣乔治呼吸问卷（SGRQ）得分较差与加重率升高或高吸烟量之间存在关联。目前缺乏关于疾病期间加重率与SGRQ恶化之间关系的纵向数据。本研究的目的不仅是提供横断面数据，还提供长达7年（中位随访期3.33年）的随访期内生活质量恶化的信息。
我们首先在横断面分析中，然后在纵向分析中，调查了德国AATD登记处基于问卷的数据，这些数据涉及SGRQ与加重频率、吸烟史、第1秒用力呼气量（FEV）和一氧化碳弥散量（DLCO）之间的关系。
868名蛋白酶抑制剂ZZ（PiZZ）基因型个体，平均年龄52.6±12.8岁，SGRQ评分为45.7±20.6。SGRQ与过去2年内的加重频率（=0.464；<0.001）、吸烟史（=0.233；<0.001）、FEV（=-0.436；<0.001）、DLCO（=-0.333；<0.001）以及患者年龄（=0.292；<0.001）显著相关。有职业性粉尘暴露的个体生活质量明显较差（<0.001）。所有有可用随访数据的患者（n=286）中，SGRQ的平均每年恶化程度为每年1.21±4.45分。单因素和多因素分析显示，随访期内SGRQ/年的恶化与加重频率之间存在显著关系（=0.144；=0.015）。
PiZZ AATD个体中SGRQ的恶化与加重频率相关。
