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Association between alpha1 antitrypsin (AAT) circulating polymers (CP) and lung and liver disease in patients with AAT deficiency (AATD)

Alexa Nuñez, Irene Belmonte, Elena Miranda, Eduardo Loeb, Miriam Barrecheguren, Georgina Farago, Esther Rodriguez, Francisco Rodriguez-Frías, Marc Miravitlles, Cristina Esquinas
European Respiratory Journal 2021 58: PA3625; DOI: 10.1183/13993003.congress-2021.PA3625
Alexa Nuñez
1Pneumology Department, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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  • For correspondence: alexagnd01@hotmail.com
Irene Belmonte
2Department of Clinical Biochemistry, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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Elena Miranda
3Department of Biology and Biotechnologies ‘Charles Darwin’ and Pasteur Institute – Cenci Bolognetti Foundation, Sapienza University of Rome, Rome, Italy
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Eduardo Loeb
1Pneumology Department, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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Miriam Barrecheguren
1Pneumology Department, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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Georgina Farago
1Pneumology Department, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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Esther Rodriguez
1Pneumology Department, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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Francisco Rodriguez-Frías
2Department of Clinical Biochemistry, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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Marc Miravitlles
1Pneumology Department, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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Cristina Esquinas
1Pneumology Department, Hospital Universitari Vall d´Hebron, Barcelona, Spain
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Abstract

AATD is characterised by the misfolding and intracellular polymerization of mutant AAT protein within hepatocytes. Polymers can also be found in peripheral blood after secretion into circulation.

This was a cross-sectional study to analyse the relationship between CP and lung and liver disease in patients with AATD. Patients with mixed phenotypes of AATD, COPD without AATD and healthy controls were included. To quantify CP, a sandwich ELISA was performed using the 2C1 monoclonal antibody against AAT polymers. Sociodemographic data, clinical characteristics, and liver and lung parameters were collected.

A cohort of 117 individuals was recruited: 32(27%) ZZ(11 on augmentation therapy);29(25%) Z-heterozygous;16(14%) with at least one S allele;5(4%) rare variants; and 35(30%) MM. ZZ patients had higher CP(p<0.001) than other phenotypes. ZZ also had higher levels of liver stiffness measurements (LSM) than the remaining AATD patients (p<0.05). ZZ and Z-heterozygous non-augmented patients were classified into 4 groups according to lung(FEV1/FVC<and>=0.7); and liver impairment(LSM < and > =6kPa). Two patients with lung and liver impairment had the highest concentrations of CP(47.5 µg/ml), followed by liver abnormality only (n=6, CP=34µg/ml), lung only (n=18, CP=26.5µg/ml) and no abnormalities(n=23, CP=14.3µg/ml). Differences were highly significant (p=0.004).

Non-augmented ZZ and Z-patients with impaired lung function and increased liver stiffness presented higher levels of CP than other clinical phenotypes. Therefore, CP may help to identify patients at higher risk of developing lung and liver disease and may provide some insight into the mechanisms of disease.

  • COPD
  • Chronic diseases

Footnotes

Cite this article as: European Respiratory Journal 2021; 58: Suppl. 65, PA3625.

This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.

This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).

  • Copyright ©the authors 2021
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Association between alpha1 antitrypsin (AAT) circulating polymers (CP) and lung and liver disease in patients with AAT deficiency (AATD)
Alexa Nuñez, Irene Belmonte, Elena Miranda, Eduardo Loeb, Miriam Barrecheguren, Georgina Farago, Esther Rodriguez, Francisco Rodriguez-Frías, Marc Miravitlles, Cristina Esquinas
European Respiratory Journal Sep 2021, 58 (suppl 65) PA3625; DOI: 10.1183/13993003.congress-2021.PA3625

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Association between alpha1 antitrypsin (AAT) circulating polymers (CP) and lung and liver disease in patients with AAT deficiency (AATD)
Alexa Nuñez, Irene Belmonte, Elena Miranda, Eduardo Loeb, Miriam Barrecheguren, Georgina Farago, Esther Rodriguez, Francisco Rodriguez-Frías, Marc Miravitlles, Cristina Esquinas
European Respiratory Journal Sep 2021, 58 (suppl 65) PA3625; DOI: 10.1183/13993003.congress-2021.PA3625
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