The Role of Neutrophils in ANCA-Associated Vasculitis: The Pathogenic Role and Diagnostic Utility of Autoantibodies

Walulik, Agata; Łysak, Kinga; Błaszkiewicz, Michał; Górecki, Ignacy; Gomułka, Krzysztof

doi:10.3390/ijms242417217

Cited by 3 publications

(3 citation statements)

References 98 publications

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“…One type, known as perinuclear ANCA (p-ANCA), is characterized by staining around the nucleus, whereas the other type, known as cytoplasmic ANCA (c-ANCA), exhibits diffuse staining of the cytoplasm, with the primary antigens targeted by p-ANCA and c-ANCA being MPO and PR3, respectively [77]. In addition to MPO and PR3, ANCAs have the potential to target various other neutrophil-derived molecules, including α-enolase, azurocidin, bactericidal permeability-increasing protein (BPI), cathepsin G, elastase, defensin, lactoferrin, lysosomeassociated membrane glycoprotein 2 (LAMP2), and moesin; however, these 'minor' ANCAs generally exhibit low pathogenicity and are not typically associated with vasculitis [78].…”

Section: Common Pathogenetic Mechanisms In Aavmentioning

confidence: 99%

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Fouka,

Drakopanagiotakis,

Steiropoulos

2024

IJMS

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Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals.

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Section: Common Pathogenetic Mechanisms In Aavmentioning

confidence: 99%

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Fouka,

Drakopanagiotakis,

Steiropoulos

2024

IJMS

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“…This disorder manifests through a breakdown in the body’s tolerance to neutrophil primary granule proteins, such as leukocyte proteinase 3 (PR3) or myeloperoxidase (MPO) [ 7 ]. AAV is clinically classified into three main types, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), each presenting distinct clinical features [ 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

“…It take into account ANCA testing and modern imaging techniques, resulting in improved specificity and sensitivity. Therefore, in each case we were able to ascertain that the developed disease was associated with ANCA antibodies, confirmed by laboratory tests and biopsy [ 9 , 11 ]. We summarized the characteristics and clinical presentation of ANCA-positive small-vessel vasculitis cases reported in the literature since the beginning of the COVID-19 pandemic.…”

Section: Introductionmentioning

confidence: 99%

ANCA-Positive Small-Vessel Vasculitis Following SARS-CoV-2 Vaccination—A Systematic Review

Łysak,

Walulik,

Błaszkiewicz

et al. 2024

Vaccines

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As vaccinations against the SARS-CoV-2 virus have become a crucial tool in controlling the spread of the disease, reports of rare health complications have emerged, including new-onset antineutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitis (AAV). We systematically reviewed new-onset AAV following COVID-19 vaccination case reports and case series published in three databases before January 2024 following PRISMA guidelines to understand the characteristics of possible causal relationships or coincidences. In total, 404 articles were screened respectively by title, abstracts, and full-texts. Thirty-four papers fulfilled the inclusion criteria and have been analyzed, covering 44 patients with new-onset AAV after COVID-19 vaccination with no prior history of COVID-19 infection. Data regarding patients’ metrics, comorbidities, vaccination characteristics, symptoms, diagnostics, treatment, and outcomes were investigated and summarized. The cohort consisted predominantly of females. AAV diagnosis was confirmed via biopsy, with renal dysfunction as a prevailing manifestation. In most cases, the first symptoms of AAV developed after the second dose; moreover, Pfizer-BioNTech was the most frequently administered vaccine among the analyzed cohort. Primary treatment involved glucocorticoid therapy, with a mostly favourable response. This systematic review aims to raise awareness among clinicians in the field regarding this rare but possible complication, to promote the prompt recognition and diagnosis of de novo ANCA-positive small-vessel vasculitis in timely association with SARS-CoV-2 vaccination.

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Serum calprotectin in vasculitis associated with antineutrophil cytoplasmic antibodies

Vorkel,

Reshetnyak,

Nurbaeva

et al. 2024

Sovremennaâ revmatologiâ

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Antineutrophil cytoplasmic antibody-associated systemic vasculitis (ANCA-SV) is a group of rare and potentially severe systemic diseases. The search for reliable methods to assess ANCA-SV activity remains relevant. Among the indicators of neutrophil activation that have emerged in clinical practice, the level of serum calprotectin (CLP) stands out, which can be a marker for monitoring vasculitis activity and identifying patients at risk of disease relapse. Objective: to determine serum CLP levels in patients with ANCA-SV. Material and methods. The study group comprised 64 patients (37 with granulomatosis with polyangiitis, 11 with eosinophilic granulomatosis with polyangiitis and 16 with microscopic polyangiitis) aged 18 years and older with a confirmed diagnosis of ANCA-SV. The control group consisted of 30 healthy individuals. ANCA-SV activity was determined using the BVAS index; high activity corresponded to a BVAS value of >3. Damage was assessed using the VDI index. Depending on ANCA-SV activity, patients were divided into two groups: high activity group (group 1, n=33) and low activity group (group 2, n=31). In addition to the generally accepted indicators, serum CLP levels were assessed in all patients with ANCA-SV and healthy donors. Results and discussion. Statistically significant differences (p<0.001) were found in CLP levels in patients with ANCA-SV in groups 1 and 2. A significant correlation was found between CLP concentration and leukocyte count, neutrophil count, neutrophil-to-lymphocyte ratio (NLR) and systemic inflammatory index (SII). Blood CLP levels in ANCA-SV were associated with creatinine levels and not with glomerular filtration rate and urinary sediment. Although CLP concentration depended on disease activity, it did not correlate with acute phase indicators, including ESR and CRP concentration. Conclusion. Serum CLP concentration is significantly higher in patients with active ANCA-SV and is related to NLR and SII inflammatory indices, so we consider the possibility of using this indicator to assess disease activity.

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The Role of Neutrophils in ANCA-Associated Vasculitis: The Pathogenic Role and Diagnostic Utility of Autoantibodies

Cited by 3 publications

References 98 publications

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

ANCA-Positive Small-Vessel Vasculitis Following SARS-CoV-2 Vaccination—A Systematic Review

Serum calprotectin in vasculitis associated with antineutrophil cytoplasmic antibodies

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