Alpha 1-antitrypsin deficiency: the radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: a survey by the British Thoracic Association

Clin Radiol. 1982 Jul;33(4):371-7. doi: 10.1016/s0009-9260(82)80297-3.


In a multicentred survey of alpha 1-antitrypsin deficiency, radiological details have been obtained in 165 subjects homozygous for Pi type Z and in 23 subjects heterozygous for type SZ. Eight-five per cent of all type Z subjects had radiological evidence of pulmonary emphysema which was commoner in smokers than in those who had never smoked and which predominantly affected the lower zones of the lungs; 66% of the type Z subjects identified through family studies also had emphysema. Emphysema in subjects of Pi type SZ was limited to those who had smoked; this phenotype may not carry a specific risk of emphysema.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Female
  • Heterozygote
  • Homozygote
  • Humans
  • Lung / diagnostic imaging
  • Male
  • Pulmonary Emphysema / diagnostic imaging*
  • Pulmonary Emphysema / genetics
  • Radiography
  • Sex Factors
  • Smoking
  • alpha 1-Antitrypsin / genetics
  • alpha 1-Antitrypsin Deficiency*


  • alpha 1-Antitrypsin