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Decreased Risk of Ischemic Heart Disease in Individuals with Severe Alpha 1-Antitrypsin Deficiency (PiZZ) in Comparison with the General Population

Authors Tanash H, Ekström M, Basil N, Rönmark E, Lindberg A, Piitulainen E

Received 27 January 2020

Accepted for publication 20 April 2020

Published 4 June 2020 Volume 2020:15 Pages 1245—1252


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Dr Richard Russell

Hanan Tanash,1 Magnus Ekström,2 Nawfal Basil,1 Eva Rönmark,3 Anne Lindberg,4 Eeva Piitulainen1

1Departments of Respiratory Medicine, Skåne University Hospital, Lund University, Lund, Sweden; 2Faculty of Medicine, Department of Clinical Sciences Lund, Respiratory Medicine and Allergology, Lund University, Lund, Sweden; 3Department of Public Health and Clinical Medicine, The OLIN Unit, Division of Occupational and Environmental Medicine, Umeå University, Umeå, Sweden; 4Department of Public Health and Clinical Medicine, Division of Medicine, Umeå University, Umeå, Sweden

Correspondence: Hanan Tanash
Department of Respiratory Medicine, Malmö S-205 02, Sweden
Tel +46 40 33 10 00
Fax +46 40 33 62 25

Background: Severe alpha-1-antitrypsin deficiency (AATD) is an established risk factor for chronic obstructive pulmonary disease (COPD) and liver disease, but the effect on the incidence of ischemic heart disease (IHD) is not well known. The aim was to evaluate the risk of incident IHD in patients with severe AATD compared with a random sample of the general population, with known smoking habits.
Methods: AAT-deficient individuals, phenotype PiZZ (n=1545), were included in the Swedish National AATD Register. Controls (n=5883) were selected from population-based cohorts in Northern Sweden. Data on IHD and comorbidities were obtained by nationwide cross-linkage with the Swedish National Patient Register. Risk factors for incident IHD were analyzed using Cox regression, adjusted for age, gender, smoking status and the presence of COPD, hypertension, hyperlipidemia and diabetes.
Results: At inclusion, 46% of the PiZZ individuals and 53% of the controls were never-smokers. During follow-up (median 16 years; range 0.2– 23), 8% (n=123) of PiZZ individuals and 12% (n=690) of controls developed IHD. The controls had a significantly higher risk for incident IHD than the PiZZ individuals, with adjusted hazard ratio (HR) of 1.8 (95% CI 1.4– 2.3). The risk was higher for controls in both ever-smokers (HR 2.1; 95% CI 1.5– 2.9) and never-smokers (HR 1.5; 95% CI 1.1– 2.2).
Conclusion: PiZZ individuals have a lower risk of developing incident ischemic heart disease than the control subjects with known smoking habits, who had been randomly selected from population-based cohorts.

Keywords: alpha-1-antitrypsin deficiency, chronic obstructive pulmonary disease, ischemic heart disease, smoking

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