Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers

Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of small-vessel vasculitides, characterized by the presence of antibodies binding to myeloperoxidase (MPO) and proteinase-3 (PR3) found in neutrophil granules. Apart from being the target of ANCA, neut...

Full description

Saved in:
Bibliographic Details
Main Authors: Anna Drynda, Marcin Surmiak, Stanisława Bazan-Socha, Katarzyna Wawrzycka-Adamczyk, Mariusz Korkosz, Jacek Musiał, Krzysztof Wójcik
Format: Article
Language:English
Published: MDPI AG 2025-07-01
Series:Diagnostics
Subjects:
Online Access:https://www.mdpi.com/2075-4418/15/15/1905
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849406268431663104
author Anna Drynda
Marcin Surmiak
Stanisława Bazan-Socha
Katarzyna Wawrzycka-Adamczyk
Mariusz Korkosz
Jacek Musiał
Krzysztof Wójcik
author_facet Anna Drynda
Marcin Surmiak
Stanisława Bazan-Socha
Katarzyna Wawrzycka-Adamczyk
Mariusz Korkosz
Jacek Musiał
Krzysztof Wójcik
author_sort Anna Drynda
collection DOAJ
description Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of small-vessel vasculitides, characterized by the presence of antibodies binding to myeloperoxidase (MPO) and proteinase-3 (PR3) found in neutrophil granules. Apart from being the target of ANCA, neutrophils actively contribute to the vicious cycle of inflammation and vascular damage in AAV. On the other hand, platelets have recently been recognized as essential for thrombosis and as inflammatory effectors that collaborate with neutrophils, reinforcing the generation of reactive oxygen species (ROS) and the formation of neutrophil extracellular traps (NETs) in those diseases. Neutrophils exhibit morphological and functional heterogeneity in AAV, reflecting the complexity of their contribution to disease pathogenesis. Since long-term immunosuppression may be related to serious infections and malignancies, there is an urgent need for reliable biomarkers of disease activity to optimize the management of AAV. This review summarizes the current understanding of the role of neutrophils and platelets in the pathogenesis of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), focusing on their crosstalk, and highlights the potential for identifying novel biomarkers relevant for predicting the disease course and its relapses.
format Article
id doaj-art-e872f4f345f241b18b3ee58cb5bd90ba
institution Kabale University
issn 2075-4418
language English
publishDate 2025-07-01
publisher MDPI AG
record_format Article
series Diagnostics
spelling doaj-art-e872f4f345f241b18b3ee58cb5bd90ba2025-08-20T03:36:26ZengMDPI AGDiagnostics2075-44182025-07-011515190510.3390/diagnostics15151905Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity BiomarkersAnna Drynda0Marcin Surmiak1Stanisława Bazan-Socha2Katarzyna Wawrzycka-Adamczyk3Mariusz Korkosz4Jacek Musiał5Krzysztof Wójcik6Doctoral School of Medical Sciences and Health Sciences, Jagiellonian University Medical College, 31-530 Cracow, Poland2nd Department of Internal Medicine, Faculty of Medicine, Jagiellonian University Medical College, 30-688 Cracow, Poland2nd Department of Internal Medicine, Faculty of Medicine, Jagiellonian University Medical College, 30-688 Cracow, Poland2nd Department of Internal Medicine, Faculty of Medicine, Jagiellonian University Medical College, 30-688 Cracow, PolandDepartment of Rheumatology and Immunology, Jagiellonian University Medical College, 30-688 Cracow, Poland2nd Department of Internal Medicine, Faculty of Medicine, Jagiellonian University Medical College, 30-688 Cracow, Poland2nd Department of Internal Medicine, Faculty of Medicine, Jagiellonian University Medical College, 30-688 Cracow, PolandAnti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of small-vessel vasculitides, characterized by the presence of antibodies binding to myeloperoxidase (MPO) and proteinase-3 (PR3) found in neutrophil granules. Apart from being the target of ANCA, neutrophils actively contribute to the vicious cycle of inflammation and vascular damage in AAV. On the other hand, platelets have recently been recognized as essential for thrombosis and as inflammatory effectors that collaborate with neutrophils, reinforcing the generation of reactive oxygen species (ROS) and the formation of neutrophil extracellular traps (NETs) in those diseases. Neutrophils exhibit morphological and functional heterogeneity in AAV, reflecting the complexity of their contribution to disease pathogenesis. Since long-term immunosuppression may be related to serious infections and malignancies, there is an urgent need for reliable biomarkers of disease activity to optimize the management of AAV. This review summarizes the current understanding of the role of neutrophils and platelets in the pathogenesis of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), focusing on their crosstalk, and highlights the potential for identifying novel biomarkers relevant for predicting the disease course and its relapses.https://www.mdpi.com/2075-4418/15/15/1905ANCA-associated vasculitis (AAV)neutrophilsplateletsmyeloperoxidase (MPO)proteinase-3 (PR3)neutrophil extracellular traps (NETs)
spellingShingle Anna Drynda
Marcin Surmiak
Stanisława Bazan-Socha
Katarzyna Wawrzycka-Adamczyk
Mariusz Korkosz
Jacek Musiał
Krzysztof Wójcik
Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers
Diagnostics
ANCA-associated vasculitis (AAV)
neutrophils
platelets
myeloperoxidase (MPO)
proteinase-3 (PR3)
neutrophil extracellular traps (NETs)
title Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers
title_full Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers
title_fullStr Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers
title_full_unstemmed Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers
title_short Neutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers
title_sort neutrophils and platelets as key players in the pathogenesis of anca associated vasculitis and potential sources of disease activity biomarkers
topic ANCA-associated vasculitis (AAV)
neutrophils
platelets
myeloperoxidase (MPO)
proteinase-3 (PR3)
neutrophil extracellular traps (NETs)
url https://www.mdpi.com/2075-4418/15/15/1905
work_keys_str_mv AT annadrynda neutrophilsandplateletsaskeyplayersinthepathogenesisofancaassociatedvasculitisandpotentialsourcesofdiseaseactivitybiomarkers
AT marcinsurmiak neutrophilsandplateletsaskeyplayersinthepathogenesisofancaassociatedvasculitisandpotentialsourcesofdiseaseactivitybiomarkers
AT stanisławabazansocha neutrophilsandplateletsaskeyplayersinthepathogenesisofancaassociatedvasculitisandpotentialsourcesofdiseaseactivitybiomarkers
AT katarzynawawrzyckaadamczyk neutrophilsandplateletsaskeyplayersinthepathogenesisofancaassociatedvasculitisandpotentialsourcesofdiseaseactivitybiomarkers
AT mariuszkorkosz neutrophilsandplateletsaskeyplayersinthepathogenesisofancaassociatedvasculitisandpotentialsourcesofdiseaseactivitybiomarkers
AT jacekmusiał neutrophilsandplateletsaskeyplayersinthepathogenesisofancaassociatedvasculitisandpotentialsourcesofdiseaseactivitybiomarkers
AT krzysztofwojcik neutrophilsandplateletsaskeyplayersinthepathogenesisofancaassociatedvasculitisandpotentialsourcesofdiseaseactivitybiomarkers