Skip to main content

Lund University Publications

LUND UNIVERSITY LIBRARIES

Can Plasma ADAMTS13 Differentiate Patients With Pulmonary Arterial Hypertension From Other Forms of Pulmonary Hypertension and Dyspnea Control Patients

Kania, Kriss LU orcid ; Ahmed, Abdulla LU orcid ; Ahmed, Salaheldin LU orcid ; Engel Sällberg, Adam LU orcid ; Tran-Lundmark, Karin LU ; Carlsen, Jørn and Rådegran, Göran LU (2025) In CHEST Pulmonary 3(3). p.1-12
Abstract

Background: Multimarker panels of blood-borne biomarkers could aid in shortening the diagnostic delay of pulmonary arterial hypertension (PAH). Research Question: Can any of the 61 proteins included in the study, related to pathways known to be involved in PAH pathophysiology (eg, coagulation, inflammation, fibrosis), differentiate PAH from other forms of pulmonary hypertension (PH) and/or dyspnea and thus aid in diagnosing PAH? Study Design and Methods: Plasma samples were collected from 55 healthy control participants and 355 patients at diagnosis, including PAH (n = 95), chronic thromboembolic PH (n = 54), heart failure with preserved ejection fraction with PH (n = 58), heart failure with reduced ejection fraction with PH (n = 64), a... (More)

Background: Multimarker panels of blood-borne biomarkers could aid in shortening the diagnostic delay of pulmonary arterial hypertension (PAH). Research Question: Can any of the 61 proteins included in the study, related to pathways known to be involved in PAH pathophysiology (eg, coagulation, inflammation, fibrosis), differentiate PAH from other forms of pulmonary hypertension (PH) and/or dyspnea and thus aid in diagnosing PAH? Study Design and Methods: Plasma samples were collected from 55 healthy control participants and 355 patients at diagnosis, including PAH (n = 95), chronic thromboembolic PH (n = 54), heart failure with preserved ejection fraction with PH (n = 58), heart failure with reduced ejection fraction with PH (n = 64), a non-PH dyspnea control group with heart failure (n = 45), and an independent external PAH cohort (n = 39) used as external validation of protein levels in the PAH group of the discovery cohort. Plasma levels of the 61 proteins were analyzed using proximity extension assay. Results: ADAMTS13 plasma levels differed in patients with PAH compared with the 3 other PH groups and the non-PH dyspnea group (P < .05). In univariable (OR, 0.90; 95% CI, 0.84-0.96) and multivariable logistic regression models adjusted for age and sex (OR, 0.89; 95% CI, 0.83-0.96), ADAMTS13 was additionally able to differentiate PAH from the other combined disease groups. In the external validation cohort, plasma ADAMTS13 levels in PAH were statistically equivalent to the discovery cohort (P < .002). Interpretation: Plasma ADAMTS13 may be a diagnostic biomarker in PAH with the ability to differentiate patients with PAH from other dyspnea groups and is an interesting protein for inclusion in future studies of multimarker panels. Further validation in larger cohorts is warranted.

(Less)
Please use this url to cite or link to this publication:
author
; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
ADAM-TS13, ADAMTS-13, diagnosis, discrimination, HFpEF, HFrEF, thrombosis
in
CHEST Pulmonary
volume
3
issue
3
article number
100178
pages
1 - 12
publisher
Elsevier
external identifiers
  • scopus:105015517081
DOI
10.1016/j.chpulm.2025.100178
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2025 The Authors
id
777a2c81-7f19-4378-b507-b0aee6510669
date added to LUP
2025-09-26 17:08:00
date last changed
2025-10-14 09:09:22
@article{777a2c81-7f19-4378-b507-b0aee6510669,
  abstract     = {{<p>Background: Multimarker panels of blood-borne biomarkers could aid in shortening the diagnostic delay of pulmonary arterial hypertension (PAH). Research Question: Can any of the 61 proteins included in the study, related to pathways known to be involved in PAH pathophysiology (eg, coagulation, inflammation, fibrosis), differentiate PAH from other forms of pulmonary hypertension (PH) and/or dyspnea and thus aid in diagnosing PAH? Study Design and Methods: Plasma samples were collected from 55 healthy control participants and 355 patients at diagnosis, including PAH (n = 95), chronic thromboembolic PH (n = 54), heart failure with preserved ejection fraction with PH (n = 58), heart failure with reduced ejection fraction with PH (n = 64), a non-PH dyspnea control group with heart failure (n = 45), and an independent external PAH cohort (n = 39) used as external validation of protein levels in the PAH group of the discovery cohort. Plasma levels of the 61 proteins were analyzed using proximity extension assay. Results: ADAMTS13 plasma levels differed in patients with PAH compared with the 3 other PH groups and the non-PH dyspnea group (P &lt; .05). In univariable (OR, 0.90; 95% CI, 0.84-0.96) and multivariable logistic regression models adjusted for age and sex (OR, 0.89; 95% CI, 0.83-0.96), ADAMTS13 was additionally able to differentiate PAH from the other combined disease groups. In the external validation cohort, plasma ADAMTS13 levels in PAH were statistically equivalent to the discovery cohort (P &lt; .002). Interpretation: Plasma ADAMTS13 may be a diagnostic biomarker in PAH with the ability to differentiate patients with PAH from other dyspnea groups and is an interesting protein for inclusion in future studies of multimarker panels. Further validation in larger cohorts is warranted.</p>}},
  author       = {{Kania, Kriss and Ahmed, Abdulla and Ahmed, Salaheldin and Engel Sällberg, Adam and Tran-Lundmark, Karin and Carlsen, Jørn and Rådegran, Göran}},
  keywords     = {{ADAM-TS13; ADAMTS-13; diagnosis; discrimination; HFpEF; HFrEF; thrombosis}},
  language     = {{eng}},
  number       = {{3}},
  pages        = {{1--12}},
  publisher    = {{Elsevier}},
  series       = {{CHEST Pulmonary}},
  title        = {{Can Plasma ADAMTS13 Differentiate Patients With Pulmonary Arterial Hypertension From Other Forms of Pulmonary Hypertension and Dyspnea Control Patients}},
  url          = {{http://dx.doi.org/10.1016/j.chpulm.2025.100178}},
  doi          = {{10.1016/j.chpulm.2025.100178}},
  volume       = {{3}},
  year         = {{2025}},
}