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Smoking, lung function, and alpha 1-antitrypsin deficiency.

E D Janus, N T Phillips, R W Carrell

    Lancet (London, England)
    |January 19, 1985
    PubMed
    Summary
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    Individuals with alpha-1-antitrypsin deficiency (ZZ) who smoke experience earlier and more severe emphysema. Smoking significantly accelerates lung function decline and reduces lifespan compared to non-smokers.

    Area of Science:

    • Pulmonology
    • Genetics
    • Internal Medicine

    Background:

    • Alpha-1-antitrypsin deficiency (ZZ) is a genetic condition predisposing individuals to early-onset emphysema.
    • Smoking is a major exacerbating factor in the progression of lung disease in ZZ individuals.

    Purpose of the Study:

    • To investigate the impact of smoking on the clinical course of emphysema in individuals with homozygous alpha-1-antitrypsin deficiency.
    • To compare disease onset, progression, and survival rates between smokers and non-smokers with ZZ deficiency.

    Main Methods:

    • Longitudinal follow-up study of 69 individuals with ZZ deficiency from infancy to 87 years old (1970-1983).
    • Data collection included age of dyspnea onset, survival, forced expiratory volume in 1 second (FEV1) measurements, and smoking history (pack years).

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    Main Results:

    • Smokers with ZZ deficiency had a significantly earlier mean age of dyspnea onset (32 years) compared to non-smokers (51 years).
    • Mean age at death was substantially lower for smokers (48 years) versus non-smokers (67 years).
    • Smokers exhibited drastically reduced FEV1 (38% predicted) and a rapid decline rate (317 ml/year) compared to non-smokers (77% predicted, 80 ml/year decline).

    Conclusions:

    • Smoking dramatically accelerates emphysema progression and reduces survival in individuals with alpha-1-antitrypsin deficiency.
    • Cessation of smoking may significantly alter the disease trajectory and improve outcomes for ZZ individuals.
    • Early diagnosis and smoking cessation counseling are critical for managing ZZ-related lung disease.