Neutrophils in ANCA-associated vasculitis: The role in the pathogenesis and promising therapeutic strategies

The review focuses on the latest data on the role of neutrophils in the pathogenesis of ANCA-associated systemic vasculitis (AAV). Numerous signaling pathways for the regulation of neutrophil function during AAV development are discussed, including the role of neutrophil proteases, neutrophil extracellular traps, the influence of complement components and intercellular interactions. The impact of extracorporeal treatments is discussed. The perspective goals of therapy are considered.

• Publication year

  2025

• Venue

  Rheumatology Science and Practice

• Publication date

  2025-08-31

• Fields of study

  Not labeled

• Identifiers
  DOI 10.47360/1995-4484-2025-338-347
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar

• No claims are published for this paper.

• No concepts are published for this paper.

• Damage-Associated Molecular Patterns as Mediators of Thrombus Formation on Dialyzer Membrane in Critically Ill Patients

  2024cited by this paper
• Risk of Relapse of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis in a Randomized Controlled Trial of Plasma Exchange and Glucocorticoids

  2024cited by this paper
• Non-invasive biomarkers of disease activity and organ damage in ANCA-associated vasculitis: a systematic review

  2024cited by this paper
• Long-term outcome of kidney function in patients with ANCA-associated vasculitis

  2024cited by this paper
• The Janus-faced nature of complement in hemodialysis: interplay between complement, inflammation, and bioincompatibility unveiling a self-amplifying loop contributing to organ damage

  2024cited by this paper
• The first clinical experience with selective DNA plasmasorption using the NucleoCapture Device in the treatment of systemic lupus erythematosus

  2024cited by this paper
• Urine and Plasma Complement Ba Levels During Disease Flares in Patients With Antineutrophil Cytoplasmic Autoantibody–Associated Vasculitis

  2023cited by this paper
• Renal Recovery for Patients with ANCA-Associated Vasculitis and Low eGFR in the ADVOCATE Trial of Avacopan.

  2023cited by this paper
• The role of NETosis in the pathogenesis of immunoinflammatory rheumatic diseases

  2023cited by this paper
• Anti-citrullinated histone monoclonal antibody CIT-013, a dual action therapeutic for neutrophil extracellular trap-associated autoimmune diseases

  2023cited by this paper
• Kidney Histopathology Can Predict Kidney Function in ANCA-Associated Vasculitides with Acute Kidney Injury Treated with Plasma Exchanges

  2022cited by this paper
• Anti-citrullinated Protein Antibody Generation, Pathogenesis, Clinical Application, and Prospects

  2022cited by this paper
• The effects of plasma exchange in patients with ANCA-associated vasculitis: an updated systematic review and meta-analysis

  2022cited by this paper
• 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

  2021cited by this paper
• Platelet CXCL4 mediates neutrophil extracellular traps formation in ANCA-associated vasculitis

  2021cited by this paper
• A proposed role of neutrophil extracellular traps and their interplay with fibroblasts in ANCA-associated vasculitis lung fibrosis.

  2021cited by this paper
• Specific Increase in Joint Neutrophil Extracellular Traps and Its Relation to Interleukin 6 in Autoimmune Arthritis

  2021cited by this paper
• The Japanese Society for Apheresis clinical practice guideline for therapeutic apheresis

  2021cited by this paper
• Cellular Adhesion Is a Controlling Factor in Neutrophil Extracellular Trap Formation Induced by Anti-Neutrophil Cytoplasmic Antibodies

  2021cited by this paper
• CXCR1 and CXCR2 Inhibition by Ladarixin Improves Neutrophil-Dependent Airway Inflammation in Mice

  2020cited by this paper
• Inhibition of Peptidyl Arginine Deiminase-4 Prevents Renal Ischemia-Reperfusion-Induced Remote Lung Injury

  2020cited by this paper
• Colchicine Inhibits Neutrophil Extracellular Trap Formation in Patients With Acute Coronary Syndrome After Percutaneous Coronary Intervention

  2020cited by this paper
• Enhanced neutrophil apoptosis accompanying myeloperoxidase release during hemodialysis

  2020cited by this paper
• Adjunctive Treatment With Avacopan, an Oral C5a Receptor Inhibitor, in Patients With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

  2020cited by this paper
• Neutrophil Activation and Neutrophil Extracellular Trap Formation in Dialysis Patients

  2020cited by this paper
• NET Formation in Dialysis: A Valuable, albeit Mysterious and Enticing Predictor of Mortality

  2020cited by this paper
• Hydroxychloroquine inhibiting neutrophil extracellular trap formation alleviates hepatic ischemia/reperfusion injury by blocking TLR9 in mice.

  2020cited by this paper
• A Novel Peptidylarginine Deiminase 4 (PAD4) Inhibitor BMS-P5 Blocks Formation of Neutrophil Extracellular Traps and Delays Progression of Multiple Myeloma

  2020cited by this paper
• FDA-approved disulfiram inhibits pyroptosis by blocking gasdermin D pore formation

  2020cited by this paper
• Late increases in dialysis initiation after a massive disaster

  2020cited by this paper
• Therapeutic ACPA inhibits NET formation: a potential therapy for neutrophil-mediated inflammatory diseases

  2020cited by this paper
• Neutrophil extracellular traps activate IL-8 and IL-1 expression in human bronchial epithelia.

  2020cited by this paper
• Efficacy and Safety of Danirixin (GSK1325756) Co-administered With Standard-of-Care Antiviral (Oseltamivir): A Phase 2b, Global, Randomized Study of Adults Hospitalized With Influenza

  2019cited by this paper
• Neutrophil extracellular traps (NET) induced by different stimuli: A comparative proteomic analysis

  2019cited by this paper
• Interleukin-1β Mediates Arterial Thrombus Formation via NET-Associated Tissue Factor

  2019cited by this paper
• Раннее выявление идиопатического легочного фиброза: дифференциальная диагностика с интерстициальными поражениями легких при системных иммуновоспалительных ревматических заболеваниях

  2019cited by this paper
• Evaluation of appropriate indications for the use of sivelestat sodium in acute respiratory distress syndrome: a retrospective cohort study

  2019cited by this paper
• IL-1β Blockade Attenuates Thrombosis in a Neutrophil Extracellular Trap-Dependent Breast Cancer Model

  2019cited by this paper
• Understanding how Fc-modification transforms a pathogenic HIT-like monoclonal antibody into a novel treatment for sepsis.

  2019cited by this paper
• Enhanced Neutrophil Extracellular Trap Formation in Acute Pancreatitis Contributes to Disease Severity and Is Reduced by Chloroquine

  2019cited by this paper
• The antidiabetic drug metformin blunts NETosis in vitro and reduces circulating NETosis biomarkers in vivo

  2018cited by this paper
• Перспективы анти-В-клеточной терапии в ревматологии

  2018cited by this paper
• Chloroquine reduces hypercoagulability in pancreatic cancer through inhibition of neutrophil extracellular traps

  2018cited by this paper
• Complement in ANCA-associated vasculitis: mechanisms and implications for management

  2017cited by this paper
• Lipocalin-2: A Master Mediator of Intestinal and Metabolic Inflammation.

  2017cited by this paper
• Protein arginine deiminase 4 inhibition is sufficient for the amelioration of collagen‐induced arthritis

  2017cited by this paper
• Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease

  2017cited by this paper
• Pharmacological targeting of peptidylarginine deiminase 4 prevents cancer-associated kidney injury in mice

  2017cited by this paper
• Recent Advances in Antabuse (Disulfiram): The Importance of its Metal-binding Ability to its Anticancer Activity.

  2017cited by this paper
• Heat stress induces ferroptosis-like cell death in plants

  2017cited by this paper
• Association of Serum Calprotectin (S100A8/A9) Level With Disease Relapse in Proteinase 3–Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

  2016cited by this paper
• Characterization of Pharmacologic and Pharmacokinetic Properties of CCX168, a Potent and Selective Orally Administered Complement 5a Receptor Inhibitor, Based on Preclinical Evaluation and Randomized Phase 1 Clinical Study

  2016cited by this paper
• Neutrophil extracellular traps - the dark side of neutrophils.

  2016cited by this paper
• Interleukin-6 in ANCA-associated vasculitis: Rationale for successful treatment with tocilizumab.

  2015cited by this paper
• Circulating ADAM17 Level Reflects Disease Activity in Proteinase-3 ANCA-Associated Vasculitis.

  2015cited by this paper
• Neutrophil activation and nucleosomes as markers of systemic inflammation in paroxysmal nocturnal hemoglobinuria: effects of eculizumab

  2015cited by this paper
• Increased levels of neutrophil extracellular trap remnants in the circulation of patients with small vessel vasculitis, but an inverse correlation to anti-neutrophil cytoplasmic antibodies during remission.

  2015cited by this paper
• PF-1355, a Mechanism-Based Myeloperoxidase Inhibitor, Prevents Immune Complex Vasculitis and Anti–Glomerular Basement Membrane Glomerulonephritis

  2015cited by this paper
• Neutrophil extracellular trap formation is associated with autophagy‐related signalling in ANCA‐associated vasculitis

  2015cited by this paper
• Possible linkage between microscopic polyangiitis and thrombosis via neutrophil extracellular traps.

  2014cited by this paper
• The immunopathology of ANCA-associated vasculitis

  2014cited by this paper
• C5a receptor (CD88) blockade protects against MPO-ANCA GN.

  2014cited by this paper
• Autoantibodies to hLAMP-2 in ANCA-negative pauci-immune focal necrotizing GN.

  2014cited by this paper
• Peptidylarginine deiminase inhibition disrupts NET formation and protects against kidney, skin and vascular disease in lupus-prone MRL/lpr mice

  2014cited by this paper
• Leukocyte and serum S100A8/S100A9 expression reflects disease activity in ANCA-associated vasculitis and glomerulonephritis

  2013cited by this paper
• NSP4 Is Stored in Azurophil Granules and Released by Activated Neutrophils as Active Endoprotease with Restricted Specificity

  2013cited by this paper
• Первый клинический опыт коррекции остаточной аметропии после кросслинкинга роговичного коллагена у пациентов с кератоконусом на эксимерлазерной установке «Микроскан Визум»

  2013influential reference
• 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.

  2013cited by this paper
• C5a and its receptors in human anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis

  2012cited by this paper
• Monocytes, neutrophils, and platelets cooperate to initiate and propagate venous thrombosis in mice in vivo

  2012cited by this paper
• Endothelium-neutrophil interactions in ANCA-associated diseases.

  2012cited by this paper
• Renal IL-17 expression in human ANCA-associated glomerulonephritis.

  2012cited by this paper
• ANCA-stimulated neutrophils release BLyS and promote B cell survival: a clinically relevant cellular process

  2011cited by this paper
• Serum Levels of BAFF and APRIL in Myeloperoxidase Anti-Neutrophil Cytoplasmic Autoantibody-Associated Renal Vasculitis: Association with Disease Activity

  2011cited by this paper
• Complement alternative pathway acts as a positive feedback amplification of neutrophil activation.

  2011cited by this paper
• Staphylococcal Alpha-Toxin Is a Strong Inducer of Interleukin-17 in Humans

  2011cited by this paper
• Neutrophil Elastase, Proteinase 3, and Cathepsin G as Therapeutic Targets in Human Diseases

  2010cited by this paper
• Nasal carriage of Staphylococcus aureus and endonasal activity in Wegener s granulomatosis as compared to rheumatoid arthritis and chronic Rhinosinusitis with nasal polyps.

  2010cited by this paper
• Increased levels of BLyS and sVCAM-1 in anti-neutrophil cytoplasmatic antibody (ANCA)-associated vasculitides (AAV).

  2010cited by this paper
• Interleukin-17-producing T helper cells in autoimmunity.

  2010cited by this paper
• Presence of neutrophil extracellular traps and antineutrophil cytoplasmic antibodies associated with vasculitides

  2009cited by this paper
• SCH527123, a novel CXCR2 antagonist, inhibits ozone-induced neutrophilia in healthy subjects

  2009cited by this paper
• Netting neutrophils in autoimmune small-vessel vasculitis

  2009cited by this paper
• C5a receptor mediates neutrophil activation and ANCA-induced glomerulonephritis.

  2009cited by this paper
• APRIL secreted by neutrophils binds to heparan sulfate proteoglycans to create plasma cell niches in human mucosa.

  2008cited by this paper
• CpG oligodeoxynucleotide stimulates production of anti-neutrophil cytoplasmic antibodies in ANCA associated vasculitis

  2008cited by this paper
• Skewed distribution of Th17 lymphocytes in patients with Wegener's granulomatosis in remission.

  2008cited by this paper
• Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis.

  2007cited by this paper
• Effects of Cytapheresis on Tumor Necrosis Factor Receptor and on Expression of CD63 in Myeloperoxidase–Antineutrophil Cytoplasmic Autoantibody‐associated Vasculitis

  2007cited by this paper
• Plasma levels of soluble interleukin 2 receptor, soluble CD30, interleukin 10 and B cell activator of the tumour necrosis factor family during follow-up in vasculitis associated with proteinase 3-antineutrophil cytoplasmic antibodies: associations with disease activity and relapse

  2006cited by this paper
• IL-18 is upregulated in the kidney and primes neutrophil responsiveness in ANCA-associated vasculitis.

  2006cited by this paper
• Treatment With Cytapheresis for Antineutrophil Cytoplasmic Antibody‐associated Renal Vasculitis and Its Effect on Anti‐inflammatory Factors

  2005cited by this paper
• Aggravation of anti-myeloperoxidase antibody-induced glomerulonephritis by bacterial lipopolysaccharide: role of tumor necrosis factor-alpha.

  2005cited by this paper
• Antineutrophil cytoplasm antibodies directed against myeloperoxidase augment leukocyte-microvascular interactions in vivo.

  2005cited by this paper
• Improvement in rheumatoid arthritis following application of an extracorporeal granulotrap column, G-1

  2004cited by this paper
• Filtration Leukocytapheresis Therapy in the Treatment of Rheumatoid Arthritis Patients Resistant To or Failed with Methotrexate

  2004cited by this paper
• Differential Involvement of the P2Y1 and P2Y12 Receptors in Platelet Procoagulant Activity

  2003cited by this paper
• G-CSF–stimulated Neutrophils Are a Prominent Source of Functional BLyS

  2003cited by this paper
• Cyclooxygenase inhibitors and the antiplatelet effects of aspirin.

  2002cited by this paper
• Serum levels of soluble adhesion molecules ICAM-1, VCAM-1 and E-selectin in patients with Wegener's granulomatosis.

  2001cited by this paper

• No citing papers are available for this paper.