Lung density associates with survival in alpha 1 antitrypsin deficient patients

Respir Med. 2016 Mar;112:81-7. doi: 10.1016/j.rmed.2016.01.007. Epub 2016 Jan 22.


Introduction: CT density correlates with quality of life (QOL) scores and impaired upper zone lung density associates with higher mortality in alpha one antitrypsin deficiency (A1ATD). We hypothesised that decline in CT densitometry would relate to survival or deterioration in QOL in A1ATD.

Methods: All augmentation naïve PiZZ patients in the UK A1ATD registry with ≥ two successive quantitative CT scans were selected. Patients were divided into groups based on CT density decline and the relationship to survival and change in QOL compared by univariate analyses and multivariate Cox regression. Analyses were performed for whole lung, upper zone and lower zone density separately. Exploratory analyses of FEV1 subgroups were conducted.

Results: 110 patients were identified; 77 had whole lung and lung zone density recorded on two CT scans, 33 patients had upper zone data only on four scans. Decline in lower zone density associated with survival, even after adjustment for baseline lung density (p = 0.048), however upper zone density and whole lung density decline did not. This difference appeared to be driven by those with FEV1 >30% predicted.

Conclusion: Rate of change in lung densitometry could predict survival in A1ATD.

Keywords: Alpha 1 antitrypsin deficiency; Chronic obstructive pulmonary disease; Quality of life; Survival.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Bronchitis, Chronic / diagnostic imaging*
  • Bronchitis, Chronic / physiopathology
  • Disease Progression
  • Female
  • Forced Expiratory Volume
  • Humans
  • Lung / diagnostic imaging*
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Proportional Hazards Models
  • Pulmonary Diffusing Capacity
  • Pulmonary Emphysema / diagnostic imaging*
  • Pulmonary Emphysema / physiopathology
  • Quality of Life
  • Registries*
  • Survival Rate
  • Tomography, X-Ray Computed
  • United Kingdom
  • Vital Capacity
  • alpha 1-Antitrypsin Deficiency / diagnostic imaging*
  • alpha 1-Antitrypsin Deficiency / mortality
  • alpha 1-Antitrypsin Deficiency / physiopathology