Esquinas Cristina, Serreri Sonia, Barrecheguren Miriam, Rodriguez Esther, Nuñez Alexa, Casas-Maldonado Francisco, Blanco Ignacio, Pirina Pietro, Lara Beatriz, Miravitlles Marc
Pneumology Department, University Hospital Vall d'Hebron, Barcelona, Spain.
Public Health, Mental, Maternal and Child Health Nursing Department, Faculty of Medicine and Health Sciences, University of Barcelona, Barcelona, Spain.
Int J Chron Obstruct Pulmon Dis. 2018 Mar 23;13:1001-1007. doi: 10.2147/COPD.S155226. eCollection 2018.
The clinical course of alpha-1 antitrypsin deficiency (AATD) is very heterogeneous. It is estimated that 60% of individuals with severe AATD (Pi*ZZ) develop emphysema. The main objective of this study was to describe the outcomes of long-term lung function in individuals with AATD-associated emphysema after at least 8 years of follow-up.
We performed a retrospective analysis of longitudinal follow-up data of AATD PiZZ patients from the Spanish registry (AATD Spanish Registry [REDAAT]). The main follow-up outcome was the annual rate of decline in forced expiratory volume in 1 second (FEV) calculated using the FEV values at baseline and in the last post-bronchodilator spirometry available.
One hundred and twenty-two AATD PiZZ patients were analyzed. The median follow-up was 11 years (interquartile range =9-14). The mean FEV decline was 28 mL/year (SD=54), with a median of 33 mL/year. Tobacco consumption (β=19.8, <0.001), previous pneumonia (β=27.8, =0.026) and higher baseline FEV% (β=0.798, =0.016) were independently related to a faster FEV decline.
In this large cohort with a long follow-up, we observed a very variable decline of FEV. However, the mean FEV decline was similar to that observed in large cohorts of smoking-related COPD. Tobacco consumption, previous pneumonia and better lung function at baseline were related to a faster decline in FEV. These results highlight the importance of early diagnosis and effective treatment.
α-1抗胰蛋白酶缺乏症（AATD）的临床病程具有很大的异质性。据估计，60%的严重AATD患者（Pi*ZZ型）会发展为肺气肿。本研究的主要目的是描述AATD相关肺气肿患者至少8年随访后的长期肺功能结果。
我们对西班牙登记处（AATD西班牙登记处[REDAAT]）的AATD PiZZ患者纵向随访数据进行了回顾性分析。主要随访结果是使用基线时和最后一次支气管扩张剂后肺活量测定可得的1秒用力呼气量（FEV）计算的年下降率。
分析了122例AATD PiZZ患者。中位随访时间为11年（四分位间距=9 - 14年）。FEV的平均下降为每年28 mL（标准差=54），中位数为每年33 mL。吸烟（β=19.8，<0.001）、既往肺炎（β=27.8，=0.026）和更高的基线FEV%（β=0.798，=0.016）与更快的FEV下降独立相关。
在这个进行了长期随访的大型队列中，我们观察到FEV下降差异很大。然而，FEV的平均下降与在大量吸烟相关慢性阻塞性肺疾病队列中观察到的相似。吸烟、既往肺炎和基线时更好的肺功能与FEV更快下降有关。这些结果突出了早期诊断和有效治疗的重要性。
