ArticleArthritis and rheumatism2010
Transcription of proteinase 3 and related myelopoiesis genes in peripheral blood mononuclear cells of patients with active Wegener's granulomatosis.
Article in Arthritis and rheumatism, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 27 citations in OpenAlex.
- Can we cure vasculitis?Current opinion in immunology · 2025Review
- The evolving landscape of vasculitis management: past, current and emerging.Rheumatology (Oxford, England) · 2025Review
- Neutrophils in the Pathogenesis of Rheumatic Diseases: Fueling the Fire.Clinical reviews in allergy & immunology · 2021Review
- ANCA autoantigen gene expression highlights neutrophil heterogeneity where expression in normal-density neutrophils correlates with ANCA-induced activation.Kidney international · 2020Article
- MultiPLIER: A Transfer Learning Framework for Transcriptomics Reveals Systemic Features of Rare Disease.Cell systems · 2019Article
- Low Density Granulocytes in ANCA Vasculitis Are Heterogenous and Hypo-Responsive to Anti-Myeloperoxidase Antibodies.Frontiers in immunology · 2019Article
- Metabolic pathways and immunometabolism in rare kidney diseases.Annals of the rheumatic diseases · 2018Article
- The role of neutrophil extracellular traps in rheumatic diseases.Nature reviews. Rheumatology · 2018Review
- Ranking metrics in gene set enrichment analysis: do they matter?BMC bioinformatics · 2017Article
- ANCA-Associated Vasculitis Pathogenesis: A Commentary.Current rheumatology reports · 2017Review
- Histone modification signature at myeloperoxidase and proteinase 3 in patients with anti-neutrophil cytoplasmic autoantibody-associated vasculitis.Clinical epigenetics · 2016Article
- Neutrophil-Related Gene Expression and Low-Density Granulocytes Associated With Disease Activity and Response to Treatment in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.Arthritis & rheumatology (Hoboken, N.J.) · 2015Article
- A myelopoiesis gene signature during remission in anti-neutrophil cytoplasm antibody-associated vasculitis does not predict relapses but seems to reflect ongoing prednisolone therapy.Clinical and experimental immunology · 2014Article
- Granuloma in ANCA-associated vasculitides: another reason to distinguish between syndromes?Current rheumatology reports · 2013Review
- Erythroid-specific transcriptional changes in PBMCs from pulmonary hypertension patients.PloS one · 2012Article
- Increased generation of TRAP expressing multinucleated giant cells in patients with granulomatosis with polyangiitis.PloS one · 2012Article
Corrections and comments
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Authors and funding
13 authors at 2 institutions in 1 country.
Funding
Abstract
objectiveWegener's granulomatosis (WG) is a systemic inflammatory disease that is associated with substantial morbidity. The aim of this study was to understand the biology underlying WG and to discover markers of disease activity that would be useful for prognosis and treatment guidance.
methodsGene expression profiling was performed using total RNA from peripheral blood mononuclear cells (PBMCs) and granulocyte fractions from 41 patients with WG and 23 healthy control subjects. Gene set enrichment analysis (GSEA) was performed to search for candidate WG-associated molecular pathways and disease activity biomarkers. Principal components analysis was used to visualize relationships between subgroups of WG patients and controls. Longitudinal changes in proteinase 3 (PR3) gene expression were evaluated using reverse transcription-polymerase chain reaction, and clinical outcomes, including remission status and disease activity, were determined using the Birmingham Vasculitis Activity Score for WG (BVAS-WG).
resultsEighty-six genes in WG PBMCs and 40 in WG polymorphonuclear neutrophils (PMNs) were significantly up-regulated relative to controls. Genes up-regulated in WG PBMCs were involved in myeloid differentiation, and these included the WG autoantigen PR3. The coordinated regulation of myeloid differentiation genes was confirmed by GSEA. The median expression values of the 86 up-regulated genes in WG PBMCs were associated with disease activity (P = 1.3 x 10(-4)), and WG patients with low-level expression of the WG signature genes showed expression profiles that were only modestly different from that in healthy controls (P = 0.07). PR3 transcription was significantly up-regulated in WG PBMCs (P = 1.3 x 10(-5), false discovery rate [FDR] 0.002), but not in WG PMNs (P = 0.03, FDR 0.28), and a preliminary longitudinal analysis showed that the fold change in PR3 RNA levels in WG PBMCs corresponded to changes in the BVAS-WG score over time.
conclusionTranscription of PR3 and related myeloid differentiation genes in PBMCs may represent novel markers of disease activity in WG.
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