Researchers found that smoking habits influence respiratory symptoms in individuals with severe (PiZZ) hereditary alpha-1 antitrypsin deficiency (AATD) at the age of 42 years, as published in the International Journal of Chronic Obstructive Pulmonary Disease.

“Similar to our previous findings from the age of 30 onwards, the ever-smokers, independent of [alpha-1 antitrypsin] level, have more respiratory symptoms as assessed by quality of life than the never-smokers,” the researchers said.

In general, ever-smoking participants had worse health status on the Visual Analogue Scale and poorer scores on Saint George’s Respiratory Questionnaire than never-smoking participants.

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Ever-smoking individuals with PiZZ had signs of chronic obstructive pulmonary disease at the age of 42 years. Moreover, asthma was more frequently reported in these individuals than in never-smokers with PiZZ or ever-smokers with normal AAT alleles (PiMM).

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Ever-smokers with PiZZ presented with signs of airway hyperinflation. They had higher residual volume (RV), total lung capacity (TLC), and RV/TLC ratios than ever-smokers with PiMM and never-smokers with PiZZ. On the other hand, some never-smoking individuals with PiZZ showed early physiological signs of emphysema.

When comparing PiZZ with PiMM, patients with PiZZ showed lower resistance in the upper, small, and total airways. Also, there was a greater proportion of PiZZ never-smokers with a forced expiratory volume in 1 second/vital capacity (FEV1/VC) ratio < 0.7 when compared to never-smokers with PiMM.

Occupational exposure to airway irritants was reported by 42% and 31% of individuals with PiZZ and individuals with PiMM, respectively. Among participants with occupational exposure to airway irritants, patients with PiZZ had lower FEV1 and VC, and a higher RV/TLC ratio than individuals with PiMM.

AATD Carriers Who Never Smoked May Have No Association With Lung Cancer

The study counted 99 individuals with PiZZ and 90 individuals with PiMM who answered questionnaires about their smoking habits, symptoms, occupations, exposures to airway irritants, and quality of life.

Most (PiZZ, 77%, and PiMM, 67%) participants had never smoked, while 23% of individuals with PiZZ and 30% of individuals with PiMM declared to be former smokers. Only a small fraction (3%) of individuals with PiMM had smoking habits at the time of the study, while no smoker was identified in the PiZZ group.


Schramm GR, Mostafavi B, Piitulainen E, Wollmer P, Tanash HA. Lung function and health status in individuals with severe alpha-1-antitrypsin deficiency at the age of 42. Int J Chron Obstruct Pulmon Dis. 2021;16:3477-3485. doi:10.2147/COPD.S335683