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

Center:

Molecular Pneumology, Hannover Medical School, Hannover, Germany / National Institute of Tuberculosis and Lung Diseases, Warsaw, Poland

City/Country:

Hannover (Germany) / Warsaw (Poland)

Start date:

January 2024

Status:

Ongoing

Contact E-mail:

Janciauskiene.Sabina@mh-hannover.de / j.chorostowska@gmail.com

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Introduction

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

Objectives

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.