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Rare Genetic Lung Condition Triples COVID Risk, Swedish Study Finds

Patients with severe alpha-1 antitrypsin deficiency—a hereditary disorder affecting roughly 1 in 3,000 people—faced significantly elevated COVID-19 risks during the pandemic, particularly those with concurrent lung disease. The finding could reshape clinical protocols for managing vulnerable populations and inform pharmaceutical development for both genetic lung disorders and respiratory infections.

Originaltitel: COVID-19 in Individuals with Severe Alpha 1-Antitrypsin Deficiency

Abstrakt

<p><strong>Background:</strong> 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.</p><p><strong>Aim:</strong> 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.</p><p><strong>Methods:</strong> 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.</p><p><strong>Results:</strong> 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 FEV<sub>1</sub> 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)).</p><p><strong>Conclusion:</strong> 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.</p>

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