Routine Blood Biomarkers and Lung Disease in Patients with Alpha-1 Antitrypsin Deficiency from the EARCO Registry

Cristina Aljama; Alexa Núñez; Cristina Esquinas; Hanan Tanash; Eva Bartošovská; Maria Torres-Duran; Alice M Turner; Carlota Rodríguez-García; Angelo Corsico; Catarina Guimarães; José Luis López-Campos; Jens-Ulrik Stæhr Jensenn; José María Hernández-Pérez; Ane Lopez-Gonzalez; Galo Granados; Marc Miravitlles; Miriam Barrecheguren; EARCO group

doi:10.1159/000548597

Routine Blood Biomarkers and Lung Disease in Patients with Alpha-1 Antitrypsin Deficiency from the EARCO Registry

Cristina Aljama, Alexa Núñez, Cristina Esquinas, Hanan Tanash, Eva Bartošovská, Maria Torres-Duran, Alice M Turner, Carlota Rodríguez-García, Angelo Corsico, Catarina Guimarães, José Luis López-Campos, Jens-Ulrik Stæhr Jensenn, José María Hernández-Pérez, Ane Lopez-Gonzalez, Galo Granados, Marc Miravitlles^*, Miriam Barrecheguren, EARCO group

^*Corresponding author af dette arbejde

Abstract

INTRODUCTION: The aim of our study was to identify routine serum biomarkers that may be related to alpha-1 antitrypsin deficiency lung disease phenotypes and severity.

METHOD: Observational, cross-sectional, multicentre study conducted in patients with a Pi*ZZ genotype. Serum biomarkers, including neutrophil/lymphocyte ratio (NLR), eosinophil/lymphocyte ratio (ELR) and platelet/lymphocyte ratio (PLR), were calculated. Data were analysed to establish possible associations between biomarkers and lung function and lung phenotypes.

RESULTS: Among the 897 patients included, 48.4% were men with a mean age of 53.9 (standard deviation 14.7) years. Patients with chronic obstructive pulmonary disease (COPD) (n = 337) had higher haemoglobin levels (15.3 mg/dL vs. 13.9 mg/dL, p < 0.001), gamma-glutamyl transferase (GGT) (50.1 IU/L vs. 35.7 IU/L, p < 0.001), eosinophils (0.22 109/L vs. 0.19 109/L, p < 0.001), NRL (2.55 vs. 1.86), PLR (132.6 vs. 119.8), and ELR (0.12 vs. 0.1) compared to those without COPD. In multivariate analysis, older age, male sex, higher haematocrit, elevated alanine transaminase and GGT levels, and a higher NRL and PLR were associated with a worse forced expiratory volume in the first second (FEV1) (%). A higher Charlson score, elevated haematocrit and white cell count, as well as increased levels of AAT, aspartate aminotransferase (AST), GGT, and PLR were associated with worse carbon monoxide transfer coefficient (KCO) (%). Exacerbations were associated with female sex, and a higher PLR.

CONCLUSION: Some blood biomarkers are increased in patients worse lung function. However, the correlations between these biomarkers and the different measures of lung function are weak, and thus, identifying a single routine biomarker that accurately predicts disease severity and progression is challenging.

Originalsprog	Engelsk
Tidsskrift	Respiration; international review of thoracic diseases
Sider (fra-til)	1-11
Antal sider	11
ISSN	0025-7931
DOI	https://doi.org/10.1159/000548597
Status	E-pub ahead of print - 30 okt. 2025

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10.1159/000548597

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Aljama, C., Núñez, A., Esquinas, C., Tanash, H., Bartošovská, E., Torres-Duran, M., Turner, A. M., Rodríguez-García, C., Corsico, A., Guimarães, C., López-Campos, J. L., Stæhr Jensenn, J.-U., Hernández-Pérez, J. M., Lopez-Gonzalez, A., Granados, G., Miravitlles, M., Barrecheguren, M., & EARCO group (2025). Routine Blood Biomarkers and Lung Disease in Patients with Alpha-1 Antitrypsin Deficiency from the EARCO Registry. Respiration; international review of thoracic diseases, 1-11. Advance online publication. https://doi.org/10.1159/000548597

Aljama, C, Núñez, A, Esquinas, C, Tanash, H, Bartošovská, E, Torres-Duran, M, Turner, AM, Rodríguez-García, C, Corsico, A, Guimarães, C, López-Campos, JL, Stæhr Jensenn, J-U, Hernández-Pérez, JM, Lopez-Gonzalez, A, Granados, G, Miravitlles, M, Barrecheguren, M & EARCO group 2025, 'Routine Blood Biomarkers and Lung Disease in Patients with Alpha-1 Antitrypsin Deficiency from the EARCO Registry', Respiration; international review of thoracic diseases, s. 1-11. https://doi.org/10.1159/000548597

@article{9f9b9e28dcc74d5bb71c087cf23b7e0f,

title = "Routine Blood Biomarkers and Lung Disease in Patients with Alpha-1 Antitrypsin Deficiency from the EARCO Registry",

abstract = "INTRODUCTION: The aim of our study was to identify routine serum biomarkers that may be related to alpha-1 antitrypsin deficiency lung disease phenotypes and severity.METHOD: Observational, cross-sectional, multicentre study conducted in patients with a Pi*ZZ genotype. Serum biomarkers, including neutrophil/lymphocyte ratio (NLR), eosinophil/lymphocyte ratio (ELR) and platelet/lymphocyte ratio (PLR), were calculated. Data were analysed to establish possible associations between biomarkers and lung function and lung phenotypes.RESULTS: Among the 897 patients included, 48.4% were men with a mean age of 53.9 (standard deviation 14.7) years. Patients with chronic obstructive pulmonary disease (COPD) (n = 337) had higher haemoglobin levels (15.3 mg/dL vs. 13.9 mg/dL, p < 0.001), gamma-glutamyl transferase (GGT) (50.1 IU/L vs. 35.7 IU/L, p < 0.001), eosinophils (0.22 109/L vs. 0.19 109/L, p < 0.001), NRL (2.55 vs. 1.86), PLR (132.6 vs. 119.8), and ELR (0.12 vs. 0.1) compared to those without COPD. In multivariate analysis, older age, male sex, higher haematocrit, elevated alanine transaminase and GGT levels, and a higher NRL and PLR were associated with a worse forced expiratory volume in the first second (FEV1) (%). A higher Charlson score, elevated haematocrit and white cell count, as well as increased levels of AAT, aspartate aminotransferase (AST), GGT, and PLR were associated with worse carbon monoxide transfer coefficient (KCO) (%). Exacerbations were associated with female sex, and a higher PLR.CONCLUSION: Some blood biomarkers are increased in patients worse lung function. However, the correlations between these biomarkers and the different measures of lung function are weak, and thus, identifying a single routine biomarker that accurately predicts disease severity and progression is challenging.",

author = "Cristina Aljama and Alexa N{\'u}{\~n}ez and Cristina Esquinas and Hanan Tanash and Eva Barto{\v s}ovsk{\'a} and Maria Torres-Duran and Turner, {Alice M} and Carlota Rodr{\'i}guez-Garc{\'i}a and Angelo Corsico and Catarina Guimar{\~a}es and L{\'o}pez-Campos, {Jos{\'e} Luis} and {St{\ae}hr Jensenn}, Jens-Ulrik and Hern{\'a}ndez-P{\'e}rez, {Jos{\'e} Mar{\'i}a} and Ane Lopez-Gonzalez and Galo Granados and Marc Miravitlles and Miriam Barrecheguren and {EARCO group}",

note = "{\textcopyright} 2025 S. Karger AG, Basel.",

year = "2025",

month = oct,

day = "30",

doi = "10.1159/000548597",

language = "English",

pages = "1--11",

journal = "Respiration; international review of thoracic diseases",

issn = "0025-7931",

publisher = "S. Karger AG",

}

TY - JOUR

T1 - Routine Blood Biomarkers and Lung Disease in Patients with Alpha-1 Antitrypsin Deficiency from the EARCO Registry

AU - Aljama, Cristina

AU - Núñez, Alexa

AU - Esquinas, Cristina

AU - Tanash, Hanan

AU - Bartošovská, Eva

AU - Torres-Duran, Maria

AU - Turner, Alice M

AU - Rodríguez-García, Carlota

AU - Corsico, Angelo

AU - Guimarães, Catarina

AU - López-Campos, José Luis

AU - Stæhr Jensenn, Jens-Ulrik

AU - Hernández-Pérez, José María

AU - Lopez-Gonzalez, Ane

AU - Granados, Galo

AU - Miravitlles, Marc

AU - Barrecheguren, Miriam

AU - EARCO group

PY - 2025/10/30

Y1 - 2025/10/30

N2 - INTRODUCTION: The aim of our study was to identify routine serum biomarkers that may be related to alpha-1 antitrypsin deficiency lung disease phenotypes and severity.METHOD: Observational, cross-sectional, multicentre study conducted in patients with a Pi*ZZ genotype. Serum biomarkers, including neutrophil/lymphocyte ratio (NLR), eosinophil/lymphocyte ratio (ELR) and platelet/lymphocyte ratio (PLR), were calculated. Data were analysed to establish possible associations between biomarkers and lung function and lung phenotypes.RESULTS: Among the 897 patients included, 48.4% were men with a mean age of 53.9 (standard deviation 14.7) years. Patients with chronic obstructive pulmonary disease (COPD) (n = 337) had higher haemoglobin levels (15.3 mg/dL vs. 13.9 mg/dL, p < 0.001), gamma-glutamyl transferase (GGT) (50.1 IU/L vs. 35.7 IU/L, p < 0.001), eosinophils (0.22 109/L vs. 0.19 109/L, p < 0.001), NRL (2.55 vs. 1.86), PLR (132.6 vs. 119.8), and ELR (0.12 vs. 0.1) compared to those without COPD. In multivariate analysis, older age, male sex, higher haematocrit, elevated alanine transaminase and GGT levels, and a higher NRL and PLR were associated with a worse forced expiratory volume in the first second (FEV1) (%). A higher Charlson score, elevated haematocrit and white cell count, as well as increased levels of AAT, aspartate aminotransferase (AST), GGT, and PLR were associated with worse carbon monoxide transfer coefficient (KCO) (%). Exacerbations were associated with female sex, and a higher PLR.CONCLUSION: Some blood biomarkers are increased in patients worse lung function. However, the correlations between these biomarkers and the different measures of lung function are weak, and thus, identifying a single routine biomarker that accurately predicts disease severity and progression is challenging.

AB - INTRODUCTION: The aim of our study was to identify routine serum biomarkers that may be related to alpha-1 antitrypsin deficiency lung disease phenotypes and severity.METHOD: Observational, cross-sectional, multicentre study conducted in patients with a Pi*ZZ genotype. Serum biomarkers, including neutrophil/lymphocyte ratio (NLR), eosinophil/lymphocyte ratio (ELR) and platelet/lymphocyte ratio (PLR), were calculated. Data were analysed to establish possible associations between biomarkers and lung function and lung phenotypes.RESULTS: Among the 897 patients included, 48.4% were men with a mean age of 53.9 (standard deviation 14.7) years. Patients with chronic obstructive pulmonary disease (COPD) (n = 337) had higher haemoglobin levels (15.3 mg/dL vs. 13.9 mg/dL, p < 0.001), gamma-glutamyl transferase (GGT) (50.1 IU/L vs. 35.7 IU/L, p < 0.001), eosinophils (0.22 109/L vs. 0.19 109/L, p < 0.001), NRL (2.55 vs. 1.86), PLR (132.6 vs. 119.8), and ELR (0.12 vs. 0.1) compared to those without COPD. In multivariate analysis, older age, male sex, higher haematocrit, elevated alanine transaminase and GGT levels, and a higher NRL and PLR were associated with a worse forced expiratory volume in the first second (FEV1) (%). A higher Charlson score, elevated haematocrit and white cell count, as well as increased levels of AAT, aspartate aminotransferase (AST), GGT, and PLR were associated with worse carbon monoxide transfer coefficient (KCO) (%). Exacerbations were associated with female sex, and a higher PLR.CONCLUSION: Some blood biomarkers are increased in patients worse lung function. However, the correlations between these biomarkers and the different measures of lung function are weak, and thus, identifying a single routine biomarker that accurately predicts disease severity and progression is challenging.

U2 - 10.1159/000548597

DO - 10.1159/000548597

M3 - Journal article

C2 - 41166536

SN - 0025-7931

SP - 1

EP - 11

JO - Respiration; international review of thoracic diseases

JF - Respiration; international review of thoracic diseases

ER -

Routine Blood Biomarkers and Lung Disease in Patients with Alpha-1 Antitrypsin Deficiency from the EARCO Registry

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