Ekström Magnus, Tanash Hanan
Department of Respiratory Medicine and Allergology, Skåne University Hospital, Lund University, Lund, Sweden.
Int J Chron Obstruct Pulmon Dis. 2017 Nov 7;12:3281-3287. doi: 10.2147/COPD.S148509. eCollection 2017.
Individuals with severe alpha-1 antitrypsin deficiency (AATD) have an increased risk of developing COPD. However, outcomes during long-term oxygen therapy (LTOT) in patients with severe AATD and hypoxemia are unknown.
This was a prospective, population-based, consecutive cohort study of patients on LTOT due to COPD in the period from January 1, 1987, to June 30, 2015, in the Swedish National Registry for Respiratory Failure (Swedevox). Severe AATD was identified using the Swedish AATD registry and confirmed by isoelectric focusing. Data on lung transplantation (LTx) were obtained from the two lung transplantation centers in Sweden. Mortality and causes of death were assessed based on the National Causes of Death Registry and analyzed using multivariable Cox regression.
A total of 14,644 patients who started LTOT due to COPD were included in this study. No patient was lost to follow up. Patients with AATD were younger, included more males and more never smokers, and had fewer comorbidities. During a median follow-up of 1.6 years (interquartile range [IQR], 2.7) on LTOT, patients without severe AATD had a higher mortality, hazard ratio [HR] 1.53 (95% CI, 1.24-1.88), adjusting for age, sex, smoking status, body mass index, performance status, level of hypoxemia, and comorbidities. Cardiovascular deaths were increased. A higher proportion of AATD patients underwent LTx, 53 (19%) vs 118 (1%). Survival after LTx was similar for AATD and non-AATD patients and was predicted by age.
In oxygen-dependent COPD, patients with severe AATD have a longer survival time on LTOT, but they have a similar prognosis after lung transplantation compared with patients without AATD.
重度α-1抗胰蛋白酶缺乏症（AATD）患者患慢性阻塞性肺疾病（COPD）的风险增加。然而，重度AATD和低氧血症患者长期氧疗（LTOT）的结局尚不清楚。
这是一项前瞻性、基于人群的连续性队列研究，研究对象为1987年1月1日至2015年6月30日期间因COPD接受LTOT的患者，数据来自瑞典国家呼吸衰竭登记处（Swedevox）。使用瑞典AATD登记处识别重度AATD，并通过等电聚焦进行确认。肺移植（LTx）数据来自瑞典的两个肺移植中心。根据国家死亡原因登记处评估死亡率和死亡原因，并使用多变量Cox回归进行分析。
本研究共纳入14644例因COPD开始LTOT的患者。无患者失访。AATD患者更年轻，男性更多，从不吸烟者更多，合并症更少。在LTOT中位随访1.6年（四分位间距[IQR]，2.7）期间，未患重度AATD的患者死亡率更高，调整年龄、性别、吸烟状况、体重指数、体能状态、低氧血症水平和合并症后，风险比[HR]为1.53（95%CI，1.24 - 1.88）。心血管死亡增加。AATD患者接受LTx的比例更高，为53例（19%），而无AATD患者为118例（1%）。AATD患者和非AATD患者LTx后的生存率相似，且由年龄预测。
在依赖氧疗的COPD患者中，重度AATD患者LTOT的生存时间更长，但与无AATD患者相比，肺移植后的预后相似。
