Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2025 Nov 1;37(6):445-456.
doi: 10.1097/BOR.0000000000001112. Epub 2025 Jul 22.

Updates in juvenile dermatomyositis: pathogenesis and therapy

Samantha L Coss  1 , Sara E Sabbagh  2 , Hanna Kim  3
Affiliations
Review

Updates in juvenile dermatomyositis: pathogenesis and therapy

Samantha L Coss et al. Curr Opin Rheumatol. .

Abstract

Purpose of review: This review provides updates on juvenile dermatomyositis pathogenesis and treatment.

Recent findings: JDM pathogenesis research updates in genetic risk factors include C4 copy number. Studies clarify myositis-specific autoantibodies' (MSA) role in disease pathogenesis and more myositis-associated antibody (MAA) clinical associations. Recent studies validate an interferon (IFN)-regulated gene score and an IFN-related monocyte surface protein marker, SIGLEC-1. Vasculopathy and mitochondrial dysfunction evidence increases, both with ties to IFN. Studies point to not only T and B cells, but monocytes, macrophages, and neutrophils as dysregulated in JDM. Regarding treatment, there are growing reports of success with therapies targeting IFN-signaling (Janus kinase inhibitors), dazukibart (anti-IFN-beta), and anifrolumab (anti-IFNAR1). Chimeric antigen receptor (CAR) T-cell therapy targeting B-cells in a growing number of adult myositis patients and one JDM patient have dramatic reports of achieving drug-free remission.

Summary: Growing evidence show genetic markers, MSA, IFN, vasculopathy, varied immune cells, and mitochondrial dysfunction having important roles in JDM pathogenesis. Some refractory patients show benefit with newer IFN pathway-targeted therapies and cellular CAR-T-cell therapy. Further collaborative research on disease pathogenesis, treatment targets, and innovate clinical trial design is needed to increase access to more efficacious treatments in JDM.

Keywords: autoantibody; interferon; juvenile dermatomyositis; mitochondria; myositis autoantibody; pathogenesis; treatment; vasculopathy.

PubMed Disclaimer

References

    1. Papadopoulou C, Chew C, Wilkinson MGL, et al. Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care. Nat Rev Rheumatol 2023; 19:343–362.
    1. Zhu C, Han Y, Byun J, et al. Genetic architecture of idiopathic inflammatory myopathies from meta-analyses. Arthritis Rheumatol 2025; 77:750–764.
    1. Rothwell S, Cooper RG, Lundberg IE, et al. Myositis Genetics Consortium. Dense genotyping of immune-related loci in idiopathic inflammatory myopathies confirms HLA alleles as the strongest genetic risk factor and suggests different genetic background for major clinical subgroups. Ann Rheum Dis 2016; 75:1558–1566.
    1. Miller FW, Chen W, O’Hanlon TP, et al. Myositis Genetics Consortium. Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes. Genes Immun 2015; 16:470–480.
    1. Rothwell S, Chinoy H, Lamb JA. Genetics of idiopathic inflammatory myopathies: insights into disease pathogenesis. Curr Opin Rheumatol 2019; 31:611–616.

LinkOut - more resources