Natural history and life expectancy in severe alpha1-antitrypsin deficiency, Pi Z

Acta Med Scand. 1978;204(5):345-51. doi: 10.1111/j.0954-6820.1978.tb08452.x.


Clinical data from 246 adult Swedish individuals with severe alpha1-antitrypsin deficiency, Pi Z, diagnosed in 1963--77, were analyzed. Primary emphysema was present in 109 cases. Of 75 Pi Z patients with other types of chronic obstructive pulmonary disease (COPD), all but 7 showed signs of emphysema. Median age at onset of dyspnoea in Pi Z smokers was 40 years, compared to 53 in non-smokers (p less than 0.001). Of the Pi Z individuals over the age of 50, 19% had a diagnosis of liver cirrhosis and 15% signs of glomerular renal damage. Of 91 deceased patients, 56 died from COPD and 12 from liver disease. A greatly reduced survival was demonstrated in Pi Z individuals, regardless of sex. Smoking Pi Z individuals had a significantly lower life expectancy than Pi Z non-smokers (p less than 0.01).

MeSH terms

  • Adult
  • Aged
  • Asthma / mortality
  • Bronchitis / mortality
  • Female
  • Humans
  • Life Expectancy
  • Liver Cirrhosis / mortality
  • Lung Diseases, Obstructive / mortality*
  • Male
  • Middle Aged
  • Phenotype
  • Pulmonary Emphysema / mortality
  • Smoking
  • Sweden
  • alpha 1-Antitrypsin Deficiency*