COVID-19 in Individuals with Severe Alpha 1-Antitrypsin Deficiency.

Background: The risk of coronavirus (COVID-19) can be affected by the presence of certain chronic conditions. It is unknown if individuals with severe hereditary alpha-1-antitrypsin deficiency (AATD) faced an increased risk of severe COVID-19 infection during the pandemic and if COPD in this population affected the risk of severe COVID-19 outcomes.

Aim: Our aim was to investigate COVID-19 outcomes in individuals with severe AATD and to identify if COPD was a risk factor for severe disease.

Methods: Between 2021-2023 we interviewed 863 individuals with severe AATD (phenotype PiZZ) included in the Swedish National AATD Registry. Details on COVID-19 outcomes were collected. Cox regression models were used to assess risk of mild and severe COVID-19 by presence of COPD.

Results: Of 863 subjects with severe AATD, 231 reported COVID-19 infection (208 mild and 23 severe COVID-19). Subjects with severe COVID-19 were older, had lower FEV₁ values, were more likely ever-smokers and had more comorbidities compared to those with mild COVID-19. Subjects with COPD had over a 5-fold increased risk of severe COVID-19 compared to those without COPD (HR 5.43 (95% CI 1.61-18.27, p=0.006). After adjusting for potential confounders including smoking habits the risk remained significant (HR 3.72 (95% CI 1.04-13.23, p=0.043)).

Conclusion: Most patients with severe AATD exhibit mild symptoms of COVID-19 infection, managing them in the community. Patients who also have COPD are at increased risk of severe COVID-19 infection.