Targeting neutrophil-driven inflammation in adult-onset still’s disease: molecular insights from gene expression profiles

Abstract Background The rarity and heterogeneity of adult-onset Still’s disease (AOSD) pose significant challenges in understanding its precise pathogenic mechanisms, developing effective treatment options, and establishing therapeutic strategies. A comprehensive analysis of gene expression profiles...

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Main Authors: In-Woon Baek, Hyoun-Ah Kim, Kyung-Su Park, Ki-Jo Kim
Format: Article
Language:English
Published: BMC 2025-07-01
Series:Arthritis Research & Therapy
Subjects:
Online Access:https://doi.org/10.1186/s13075-025-03598-5
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author In-Woon Baek
Hyoun-Ah Kim
Kyung-Su Park
Ki-Jo Kim
author_facet In-Woon Baek
Hyoun-Ah Kim
Kyung-Su Park
Ki-Jo Kim
author_sort In-Woon Baek
collection DOAJ
description Abstract Background The rarity and heterogeneity of adult-onset Still’s disease (AOSD) pose significant challenges in understanding its precise pathogenic mechanisms, developing effective treatment options, and establishing therapeutic strategies. A comprehensive analysis of gene expression profiles could help to bridge the knowledge gaps in those areas. Methods A blood transcriptomic dataset comprising 31 patients with AOSD and 22 healthy controls was fetched. Cellular and molecular features were identified by analyzing differentially expressed genes (DEGs) and functional enrichment. Optimal molecular targets for neutrophil activation were identified using kernel-based diffusion scoring techniques. Results Blood molecular signatures indicate that neutrophil degranulation is the most enriched pathological process in AOSD. Neutrophil degranulation correlated significantly with the expression of Fcγ receptors, IL-1 receptors, and chemokine receptors and their signaling activities. IL-1 inhibitors and IL-6 inhibitors did not exhibit a diffusion score favorable for directly deactivating neutrophil degranulation, but agents targeting CXCR1/CXCR2, C5AR1, neutrophil elastase, SRC, and SYK demonstrated significant diffusion scores for neutrophil degranulation. In particular, CXCR1, CXCR2, and C5AR1 were the DEGs predominantly expressed in neutrophils and closely associated with neutrophil degranulation in a context-specific functional analysis. Conclusions Neutrophil activation is a key pathological module in AOSD. Therapeutic approaches aimed at neutrophils could offer a promising opportunity to regulate the inflammatory response in AOSD.
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spelling doaj-art-df4db525f2be46bfa6cd5d3577790ead2025-08-20T03:37:38ZengBMCArthritis Research & Therapy1478-63622025-07-0127111310.1186/s13075-025-03598-5Targeting neutrophil-driven inflammation in adult-onset still’s disease: molecular insights from gene expression profilesIn-Woon Baek0Hyoun-Ah Kim1Kyung-Su Park2Ki-Jo Kim3Division of Rheumatology, Department of Internal Medicine, College of Medicine, Ewha Womans UniversityDepartment of Rheumatology, Ajou University School of MedicineDivision of Rheumatology, Department of Internal Medicine, College of Medicine, St. Vincent’s Hospital, The Catholic University of KoreaDivision of Rheumatology, Department of Internal Medicine, College of Medicine, St. Vincent’s Hospital, The Catholic University of KoreaAbstract Background The rarity and heterogeneity of adult-onset Still’s disease (AOSD) pose significant challenges in understanding its precise pathogenic mechanisms, developing effective treatment options, and establishing therapeutic strategies. A comprehensive analysis of gene expression profiles could help to bridge the knowledge gaps in those areas. Methods A blood transcriptomic dataset comprising 31 patients with AOSD and 22 healthy controls was fetched. Cellular and molecular features were identified by analyzing differentially expressed genes (DEGs) and functional enrichment. Optimal molecular targets for neutrophil activation were identified using kernel-based diffusion scoring techniques. Results Blood molecular signatures indicate that neutrophil degranulation is the most enriched pathological process in AOSD. Neutrophil degranulation correlated significantly with the expression of Fcγ receptors, IL-1 receptors, and chemokine receptors and their signaling activities. IL-1 inhibitors and IL-6 inhibitors did not exhibit a diffusion score favorable for directly deactivating neutrophil degranulation, but agents targeting CXCR1/CXCR2, C5AR1, neutrophil elastase, SRC, and SYK demonstrated significant diffusion scores for neutrophil degranulation. In particular, CXCR1, CXCR2, and C5AR1 were the DEGs predominantly expressed in neutrophils and closely associated with neutrophil degranulation in a context-specific functional analysis. Conclusions Neutrophil activation is a key pathological module in AOSD. Therapeutic approaches aimed at neutrophils could offer a promising opportunity to regulate the inflammatory response in AOSD.https://doi.org/10.1186/s13075-025-03598-5Adult-onset still’s diseaseNeutrophil activationInterleukin-1Chemokine receptors
spellingShingle In-Woon Baek
Hyoun-Ah Kim
Kyung-Su Park
Ki-Jo Kim
Targeting neutrophil-driven inflammation in adult-onset still’s disease: molecular insights from gene expression profiles
Arthritis Research & Therapy
Adult-onset still’s disease
Neutrophil activation
Interleukin-1
Chemokine receptors
title Targeting neutrophil-driven inflammation in adult-onset still’s disease: molecular insights from gene expression profiles
title_full Targeting neutrophil-driven inflammation in adult-onset still’s disease: molecular insights from gene expression profiles
title_fullStr Targeting neutrophil-driven inflammation in adult-onset still’s disease: molecular insights from gene expression profiles
title_full_unstemmed Targeting neutrophil-driven inflammation in adult-onset still’s disease: molecular insights from gene expression profiles
title_short Targeting neutrophil-driven inflammation in adult-onset still’s disease: molecular insights from gene expression profiles
title_sort targeting neutrophil driven inflammation in adult onset still s disease molecular insights from gene expression profiles
topic Adult-onset still’s disease
Neutrophil activation
Interleukin-1
Chemokine receptors
url https://doi.org/10.1186/s13075-025-03598-5
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