Knowledge about the natural history of severe alpha 1-antitrypsin (AAT) deficiency (PiZZ) is limited. Our aim was to compare the survival of PiZZ individuals with randomly selected controls from the Swedish general population. The PiZZ subjects (n=1585) were selected from the Swedish National AATD Register. The controls (n=5999) were randomly selected from the Swedish population register. Smoking habits were known for all subjects. Median follow-up times for the PiZZ subjects (731 never-smokers) and controls (3179 never-smokers) were 12 and 17 years, respectively (p<0.001). During follow-up, 473 PiZZ subjects (30%), and 747 controls (12%) died. The PiZZ subjects had a significantly shorter survival time than the controls, p<0.001. After adjustment for gender, age, smoking habits and presence of respiratory symptoms, the risk of death was still significantly higher for the PiZZ individuals than for the controls, hazard ratio (HR) 3.2 (95% CI 2.8–3.6; p<0.001). By contrast, the risk of death was not increased in never-smoking PiZZ individuals identified by screening, compared to never-smoking controls, HR 1.2 (95% CI 0.6–2.2). The never-smoking PiZZ individuals identified by screening had a similar life expectancy to the never-smokers in the Swedish general population. Early diagnosis of AAT deficiency is of utmost importance. Early diagnosis of AAT deficiency is of utmost importance http://ow.ly/5iHm30dKYqN

中文翻译：

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与已知吸烟习惯的普通人群相比，患有严重 α1-抗胰蛋白酶缺乏症 (PiZZ) 的个体的存活率

关于严重 α1-抗胰蛋白酶 (AAT) 缺乏症 (PiZZ) 的自然病程的知识是有限的。我们的目的是将 PiZZ 个体的存活率与从瑞典一般人群中随机选择的对照进行比较。PiZZ 受试者 (n=1585) 选自瑞典国家 AATD 登记册。对照 (n=5999) 从瑞典人口登记册中随机选择。所有受试者都知道吸烟习惯。PiZZ 受试者（731 名从不吸烟者）和对照组（3179 名从不吸烟者）的中位随访时间分别为 12 年和 17 年（p<0.001）。在随访期间，473 名 PiZZ 受试者 (30%) 和 747 名对照组 (12%) 死亡。PiZZ 受试者的存活时间明显短于对照组，p<0.001。在调整性别、年龄、吸烟习惯和呼吸道症状后，PiZZ 个体的死亡风险仍然显着高于对照组，风险比 (HR) 3.2（95% CI 2.8-3.6；p<0.001）。相比之下，与从不吸烟的对照组相比，通过筛查确定的从不吸烟的 PiZZ 个体的死亡风险并未增加，HR 1.2（95% CI 0.6-2.2）。通过筛查确定的从不吸烟的 PiZZ 个体的预期寿命与瑞典一般人群中从不吸烟的人相似。AAT缺乏症的早期诊断至关重要。AAT 缺乏症的早期诊断至关重要 http://ow.ly/5iHm30dKYqN 与从不吸烟的对照组相比，HR 1.2 (95% CI 0.6–2.2)。通过筛查确定的从不吸烟的 PiZZ 个体的预期寿命与瑞典一般人群中从不吸烟的人相似。AAT缺乏症的早期诊断至关重要。AAT 缺乏症的早期诊断至关重要 http://ow.ly/5iHm30dKYqN 与从不吸烟的对照组相比，HR 1.2 (95% CI 0.6–2.2)。通过筛查确定的从不吸烟的 PiZZ 个体的预期寿命与瑞典一般人群中从不吸烟的人相似。AAT缺乏症的早期诊断至关重要。AAT 缺乏症的早期诊断至关重要 http://ow.ly/5iHm30dKYqN