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Research Project

Relationships between Z-alpha1 antitrypsin levels and routine blood biomarkers of systemic inflammation and lung function in patients Pi*ZZ

Principal Investigator:
Sabina Janciauskiene / Joanna Chorostowska- Wynimko
Molecular Pneumology, Hannover Medical School, Hannover, Germany / National Institute of Tuberculosis and Lung Diseases, Warsaw, Poland
Hannover (Germany) / Warsaw (Poland)
Start date:
January 2024
Contact E-mail:
Janciauskiene.Sabina@mh-hannover.de / j.chorostowska@gmail.com
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AAT is responsible for more than 90% of human anti-protease activity, therefore lung inflammation in Pi*ZZ patients is predominantly driven by neutrophil proteases and other inflammatory molecules.  In fact, serum biomarkers such as c-reactive protein, fibrinogen, lymphocyte counts, neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, and others have been studied to determine their association with the severity and prognosis of emphysema.

Low levels of AAT are clearly linked with emphysema phenotype of Pi*ZZ patients. However, these low basal AAT values exhibit individual variation, which per se might reflect systemic inflammatory status and disease progression


  • To determine the relationship between basal Pi*ZZ AAT levels and serum biomarkers of systemic inflammation and clinical phenotypes: emphysema, bronchiectasies, asthma and liver disease.
  • To investigate the association between basal Pi*ZZ AAT levels and serum biomarkers of systemic inflammation and the severity of lung disease measured by FEV1 and KCO in patients Pi*ZZ.
  • To investigate if basal Pi*ZZ AAT levels influence above mentioned associations with augmentation therapy.

Inclusion criteria

  • Retrospective study performed in data from EARCO International registry of patients Pi*ZZ.

Brief summary

  • First, a descriptive analysis will be performed of patients Pi*ZZ, patients will be separated into two subgroups based on the basal Pi*ZZ AAT levels.
  • Next, comparisons of serum biomarkers between patients Pi*ZZ subgroups with/ without liver and/or lung disease will be conducted.
  • Correlations between serum biomarkers and FEV1, KCO and LSM will be analysed.
  • Associations between biomarkers and clinical phenotypes such as emphysema, bronchiectasies, asthma and liver disease will be analysed in two subgroups of PI*ZZ patients.
  • Finally, above mentioned associations with augmentation therapy will be tested in two subgroups of Pi*ZZ patients.