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Lung Function and CT Densitometry in Subjects with alpha-1-Antitrypsin Deficiency and Healthy Controls at 35 Years of

Eeva Piitulainen1, Laura Cano Montero, Meltem Nystedt-Düzakin

  • 11Departments of Respiratory Medicine and Allergology, Skåne University Hospital, Lund University , Malmö , Sweden.

COPD
|October 4, 2014
PubMed

Insights

Alpha-1-antitrypsin (AAT) deficiency in never-smokers shows no emphysema signs at age 35. However, AAT deficiency individuals who smoke show early emphysema indicators, highlighting smoking

Area of Science:

  • Pulmonary Medicine
  • Genetics
  • Radiology

Background:

  • Alpha-1-antitrypsin (AAT) deficiency is a known genetic risk factor for developing pulmonary emphysema.
  • A nationwide screening of Swedish newborns in 1972-1974 identified individuals with AAT deficiency.

Purpose of the Study:

  • To assess for early signs of emphysema in individuals with PiZZ and PiSZ AAT deficiency genotypes at 35 years of age.
  • To investigate the role of smoking as a modifier in the development of emphysema in AAT-deficient individuals.

Main Methods:

  • Pulmonary function tests (PFTs) and CT densitometry were performed on study participants.
  • Key metrics analyzed included the fifteenth percentile density (PD15) and relative area below -910 HU (RA-910).
  • Comparison was made between AAT-deficient genotypes (PiZZ, PiSZ) and PiMM control subjects, stratified by smoking status.

Main Results:

  • Never-smoking AAT-deficient individuals (PiZZ, PiSZ) exhibited normal lung function compared to never-smoking controls (PiMM).
  • PiZZ ever-smokers demonstrated significantly lower carbon monoxide transfer coefficient (KCO) compared to PiSZ and PiMM never-smokers.
  • CT densitometry revealed significantly lower PD15 in PiZZ ever-smokers compared to ever-smoking controls, suggesting early emphysematous changes.

Conclusions:

  • AAT deficiency alone does not appear to cause significant lung function impairment or emphysema in never-smokers by age 35.
  • Smoking exacerbates the effects of AAT deficiency, with PiZZ ever-smokers showing potential early signs of emphysema.
  • These findings underscore the critical role of smoking cessation in managing AAT deficiency to prevent or delay the onset of pulmonary emphysema.

Keywords:
COPDCT densitometryalpha-1-antitrypsin deficiencylung function

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