Bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activation

Background: Bradykinin (BK), a 9–amino acid peptide, is a key mediator responsible for angioedema attacks in hereditary angioedema due to C1INH deficiency (HAE-C1INH). Current BK assays lack clinical utility due to the instability of BK (half-life < 30 seconds) and low pg/mL baseline levels. Obje...

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Main Authors: Jinguo Chen, MD, Yunkou Wu, PhD, J. Joanna Yu, MD, PhD, Joseph Chiao, MD, Jing Yu, PhD, Mark D. Scarupa, MD, Lili Wan, PhD, H. Henry Li, MD, PhD
Format: Article
Language:English
Published: Elsevier 2025-08-01
Series:Journal of Allergy and Clinical Immunology: Global
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Online Access:http://www.sciencedirect.com/science/article/pii/S2772829325001067
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author Jinguo Chen, MD
Yunkou Wu, PhD
J. Joanna Yu, MD, PhD
Joseph Chiao, MD
Jing Yu, PhD
Mark D. Scarupa, MD
Lili Wan, PhD
H. Henry Li, MD, PhD
author_facet Jinguo Chen, MD
Yunkou Wu, PhD
J. Joanna Yu, MD, PhD
Joseph Chiao, MD
Jing Yu, PhD
Mark D. Scarupa, MD
Lili Wan, PhD
H. Henry Li, MD, PhD
author_sort Jinguo Chen, MD
collection DOAJ
description Background: Bradykinin (BK), a 9–amino acid peptide, is a key mediator responsible for angioedema attacks in hereditary angioedema due to C1INH deficiency (HAE-C1INH). Current BK assays lack clinical utility due to the instability of BK (half-life < 30 seconds) and low pg/mL baseline levels. Objective: We sought to develop a novel method to overcome the issues in current protease inhibitor–based methods for measuring endogenous BK. Methods: Blood samples from subjects with HAE-C1INH and healthy volunteers were collected and subjected to cold activation for the contact system. Cold-induced BK was measured via LC-MS/MS. The protocol was developed and validated according to the US Food and Drug Administration Bioanalytical Method Validation guidelines. The procedure was optimized on specimen selection, collection, and process, as well as variable controls such as time windows, temperature, and storage conditions. Results: EDTA whole blood samples without protease inhibitor were incubated at 4°C for 1 and 3 days. Total BK levels increased more than 100-fold in attack-free HAE-C1INH subjects compared with healthy volunteers (324.3 ± 54.7 ng/mL [n = 33] vs 2.3 ± 0.3 ng/mL [n = 43]; mean ± SEM, P < .001). The diagnostic sensitivity and specificity were 90.9% and 97.1%, respectively. BK levels highly correlated with plasma kallikrein activity in the same samples. Conclusions: Whole blood under cold activation showed striking elevation of BK levels in subjects with HAE-C1INH, while minimally affecting healthy volunteers. The assay has been assessed for accuracy, precision and stability. It may serve as a reliable tool for HAE diagnosis and management.
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spelling doaj-art-5a64d2fdf4e74385a6c5de76c2955fcd2025-08-20T03:57:39ZengElsevierJournal of Allergy and Clinical Immunology: Global2772-82932025-08-014310050510.1016/j.jacig.2025.100505Bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activationJinguo Chen, MD0Yunkou Wu, PhD1J. Joanna Yu, MD, PhD2Joseph Chiao, MD3Jing Yu, PhD4Mark D. Scarupa, MD5Lili Wan, PhD6H. Henry Li, MD, PhD7Virant Diagnostics, Wheaton, MdVirant Diagnostics, Wheaton, MdVirant Diagnostics, Wheaton, MdVirant Diagnostics, Wheaton, MdAngioedema Center of Reference and Excellence, Institute for Asthma and Allergy, Wheaton, MdAngioedema Center of Reference and Excellence, Institute for Asthma and Allergy, Wheaton, MdVirant Diagnostics, Wheaton, Md; Angioedema Center of Reference and Excellence, Institute for Asthma and Allergy, Wheaton, MdVirant Diagnostics, Wheaton, Md; Angioedema Center of Reference and Excellence, Institute for Asthma and Allergy, Wheaton, Md; Corresponding author: H. Henry Li, MD, PhD, Institute for Asthma and Allergy, 11002 Veirs Mill Road, Suite 414, Wheaton, MD 20902.Background: Bradykinin (BK), a 9–amino acid peptide, is a key mediator responsible for angioedema attacks in hereditary angioedema due to C1INH deficiency (HAE-C1INH). Current BK assays lack clinical utility due to the instability of BK (half-life < 30 seconds) and low pg/mL baseline levels. Objective: We sought to develop a novel method to overcome the issues in current protease inhibitor–based methods for measuring endogenous BK. Methods: Blood samples from subjects with HAE-C1INH and healthy volunteers were collected and subjected to cold activation for the contact system. Cold-induced BK was measured via LC-MS/MS. The protocol was developed and validated according to the US Food and Drug Administration Bioanalytical Method Validation guidelines. The procedure was optimized on specimen selection, collection, and process, as well as variable controls such as time windows, temperature, and storage conditions. Results: EDTA whole blood samples without protease inhibitor were incubated at 4°C for 1 and 3 days. Total BK levels increased more than 100-fold in attack-free HAE-C1INH subjects compared with healthy volunteers (324.3 ± 54.7 ng/mL [n = 33] vs 2.3 ± 0.3 ng/mL [n = 43]; mean ± SEM, P < .001). The diagnostic sensitivity and specificity were 90.9% and 97.1%, respectively. BK levels highly correlated with plasma kallikrein activity in the same samples. Conclusions: Whole blood under cold activation showed striking elevation of BK levels in subjects with HAE-C1INH, while minimally affecting healthy volunteers. The assay has been assessed for accuracy, precision and stability. It may serve as a reliable tool for HAE diagnosis and management.http://www.sciencedirect.com/science/article/pii/S2772829325001067Hereditary angioedemaangioedemabradykininplasma kallikreincontact systemcold activation
spellingShingle Jinguo Chen, MD
Yunkou Wu, PhD
J. Joanna Yu, MD, PhD
Joseph Chiao, MD
Jing Yu, PhD
Mark D. Scarupa, MD
Lili Wan, PhD
H. Henry Li, MD, PhD
Bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activation
Journal of Allergy and Clinical Immunology: Global
Hereditary angioedema
angioedema
bradykinin
plasma kallikrein
contact system
cold activation
title Bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activation
title_full Bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activation
title_fullStr Bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activation
title_full_unstemmed Bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activation
title_short Bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activation
title_sort bradykinin measurement by liquid chromatography tandem mass spectrometry in subjects with hereditary angioedema enhanced by cold activation
topic Hereditary angioedema
angioedema
bradykinin
plasma kallikrein
contact system
cold activation
url http://www.sciencedirect.com/science/article/pii/S2772829325001067
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