Evidence map›Paper›PMID 42500994›Full record

ArticleRheumatology (Oxford, England)2026

IFN-related gene expression defines disease activity, organ involvement and treatment response in JDM.

Helena Codes-Méndez, Senne Cuyx, Aris E Syntakas, Afroditi Barmpakou, Elena Moraitis, Sandrine Compeyrot-Lacassagne, Muthana Al-Obaidi, Charalampia Papadopoulou

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Article in Rheumatology (Oxford, England), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Helena Codes-MéndezRheumatology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.ORCID 0000-0002-9883-9845
Senne CuyxPaediatric Rheumatology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.ORCID 0000-0002-6473-8966
Aris E SyntakasPaediatric Rheumatology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.ORCID 0009-0004-6225-9373
Afroditi BarmpakouPaediatric Rheumatology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.ORCID 0009-0005-6549-3859
Elena MoraitisPaediatric Rheumatology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.ORCID 0009-0009-9257-9753
Sandrine Compeyrot-LacassagnePaediatric Rheumatology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.ORCID 0000-0001-5533-7493
Muthana Al-ObaidiPaediatric Rheumatology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.ORCID 0000-0002-2144-3682
Charalampia PapadopoulouPaediatric Rheumatology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.ORCID 0000-0002-1237-0557

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesTo evaluate the relationship between IFN-related gene (IRG) expression and disease activity, organ involvement and serological subgroups in JDM and to assess its potential as a biomarker for disease monitoring and treatment response.

methodsPatients with JDM enrolled in a single-centre cohort were assessed using standardized clinical measures, including physician's global assessment (PGA), Childhood Myositis Assessment Scale (CMAS), Manual Muscle Testing (MMT8) and modified skin disease activity score. Associations between IRG expression and clinical features were analysed using correlation analyses and mixed-effects models. Longitudinal trajectories of disease activity were explored using latent class mixed models.

resultsSeventy-four patients contributed 158 samples. Expression of type-I IRGs (IFI27, IFI44L, IFIT1, RSAD2, SIGLEC1) and CXCL10 was significantly higher in active disease and correlated with muscle and skin disease activity. IRG expression inversely correlated with CMAS and MMT8 and positively with mDAS. Distinct organ-specific associations were identified: CXCL9 expression was associated with interstitial lung disease, while IL-18 was associated with calcinosis, cutaneous ulceration and gastrointestinal involvement. Higher IRG expression was observed in patients with anti-MDA5 and anti-NXP2 autoantibodies. Longitudinal analyses demonstrated that IRG expression paralleled disease activity trajectories and decreased in patients achieving inactive disease, including those treated with baricitinib.

conclusionIFN pathway activation is closely associated with disease activity and organ involvement in JDM. IRG expression reflects dynamic changes in disease status and may serve as a clinically useful biomarker for monitoring disease activity, stratifying patients by organ involvement and supporting response assessment to targeted therapies.

Indexed as

DermatomyositisInterferonsAdaptor Proteins, Signal TransducingAdolescentAntigensBiomarkersCarrier ProteinsChemokine CXCL10Chemokine CXCL9ChildChild, PreschoolCytoskeletal ProteinsFemaleGene ExpressionHumansInterleukin-18Adaptor Proteins, Signal TransducingAntigensBiomarkersCarrier ProteinsChemokine CXCL10Chemokine CXCL9CXCL10 protein, humanCXCL9 protein, humanCytoskeletal ProteinsIFI27 protein, humanIFI44L protein, humanIFI44 protein, humanIFIT1 protein, humanInterferonsInterleukin-18Intracellular Signaling Peptides and ProteinsMembrane ProteinsProteinsRNA-Binding ProteinsTumor Suppressor Proteinsbiomarkersdisease activityIFN type-IJDMorgan damageseverity of illness index

Identifiers

PMID42500994
PMCPMC13428460

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.